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