Overview
Congenital pancreatic cyst is a rare condition in which one or more fluid-filled sacs (cysts) form in the pancreas before birth. The pancreas is an organ located behind the stomach that helps with digestion and blood sugar control. These cysts are present from the time a baby is born, though they may not always be discovered right away. In some cases, the cysts are found during prenatal ultrasound or during imaging done in infancy or childhood for other reasons. The size and number of cysts can vary widely. Small cysts may cause no symptoms at all and may be discovered incidentally. Larger cysts can press on nearby organs and cause abdominal pain, feeding difficulties, nausea, vomiting, or a noticeable lump in the belly. In some cases, the cysts may interfere with the normal function of the pancreas, potentially affecting digestion or, less commonly, blood sugar regulation. Treatment depends on the size of the cyst, whether it causes symptoms, and whether complications develop. Small, asymptomatic cysts may simply be monitored with regular imaging. Larger or symptomatic cysts may require drainage or surgical removal. The outlook is generally good, especially when the cyst is isolated and not part of a broader syndrome. However, ongoing follow-up is important to watch for cyst growth or complications. Congenital pancreatic cysts may also sometimes occur as part of other conditions, such as Beckwith-Wiedemann syndrome or von Hippel-Lindau disease, so doctors may evaluate for associated features.
Key symptoms:
Abdominal pain or discomfortA lump or swelling felt in the abdomenNausea or vomitingFeeding difficulties in infantsPoor weight gain or failure to thriveBloating or feeling of fullnessJaundice (yellowing of the skin or eyes) if the cyst presses on the bile ductDigestive problems such as loose or fatty stoolsNo symptoms at all (found incidentally on imaging)
Clinical phenotype terms (6)— hover any for plain English
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
FDA & Trial Timeline
1 eventIcahn School of Medicine at Mount Sinai
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Congenital pancreatic cyst.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Congenital pancreatic cyst.
Community
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Start the conversation →Latest news about Congenital pancreatic cyst
Disease timeline:
New recruiting trial: Pancreas Registry and High Risk Registry
A new clinical trial is recruiting patients for Congenital pancreatic cyst
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is the size and exact location of my child's pancreatic cyst?,Does this cyst need treatment now, or can we safely monitor it?,Could this cyst be part of a genetic syndrome, and should we do genetic testing?,What symptoms should I watch for that would require urgent medical attention?,How often will my child need follow-up imaging?,If surgery is needed, what type of procedure would be recommended and what are the risks?,Will this cyst affect my child's digestion or blood sugar levels in the long term?
Common questions about Congenital pancreatic cyst
What is Congenital pancreatic cyst?
Congenital pancreatic cyst is a rare condition in which one or more fluid-filled sacs (cysts) form in the pancreas before birth. The pancreas is an organ located behind the stomach that helps with digestion and blood sugar control. These cysts are present from the time a baby is born, though they may not always be discovered right away. In some cases, the cysts are found during prenatal ultrasound or during imaging done in infancy or childhood for other reasons. The size and number of cysts can vary widely. Small cysts may cause no symptoms at all and may be discovered incidentally. Larger cy
At what age does Congenital pancreatic cyst typically begin?
Typical onset of Congenital pancreatic cyst is neonatal. Age of onset can vary across affected individuals.
Are there clinical trials for Congenital pancreatic cyst?
Yes — 1 recruiting clinical trial is currently listed for Congenital pancreatic cyst on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Congenital pancreatic cyst?
6 specialists and care centers treating Congenital pancreatic cyst are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.