Caroli's syndrome is included in the fibropolycystic abnormalities of the bile ducts. The disease is usually manifested by episodes of cholangitis. We report a case which presented as acute pancreatitis in the adulthood. The diagnosis was obtained by ultrasonography, CT scan, ERCP and liver biopsy. A choledochojejunostomy was performed with poor outcome and the patient is in the waiting list for liver transplantation.
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