Results: A previously healthy 11-year old female with a two year history of intermittent sharp epigastric pain was found to have malrotation on barium examination of the upper gastrointestinal tract. At the time of laparoscopic surgical repair, her liver was noted to be grossly cirrhotic. Post-operatively, she developed jaundice and pruritus. On exam, she was jaundiced and had splenomegaly. Her total bilirubin was 14.8 mg/dL, with a conjugated fraction of 6.6 mg/dL, ALT was 75 U/L, AST was 98 U/L, alkaline phosphatase was 590 U/L, albumin was 2.3 g/dL, and INR was 1.22. Evaluation for chronic liver diseases was performed and negative. White blood count was 5.3 k/uL, hemoglobin was 12.3 g/dL, and platelets were 85/uL. She developed ascites and a right-sided pleural effusion; cell count and culture were not suggestive of infection. Abdominal ultrasound demonstrated cirrhosis, splenomegaly, dilated extrahepatic biliary ducts, and portal hypertension. MRCP showed a grossly dilated extrahepatic biliary tree, as well as marked intrahepatic biliary dilation, compatible with a choledochal, or biliary cyst. ERCP was performed, showing a massively dilated biliary tree, with an aberrant low takeoff of the cystic duct and distal stenosis of the common bile duct. Two stents were placed with relief of her obstruction. Her ascitic pleural effusion and ankle edema were controlled with diuretic therapy. After the procedure, her bilirubin showed a downward trend, but she developed recurrent episodes of cholangitis. Repeat liver biopsy demonstrated cirrhosis, with proliferation of bile ducts and neutrophilic infiltration, consistent with biliary cyst and ascending cholangitis. Due to her recurrent symptoms and underlying cirrhosis, she underwent liver transplantation. Conclusion: Congential biliary cysts are irregular dilations of the bile ducts which are due to an abnormality in the ductal wall, possibly ductal stricture and thought to result from pancreaticobiliary malunion, which allows reflux of pancreatic juice into the bile duct, that can cause chemical and inflammatory changes in the duct wall, leading to weakness and dilation. To avoid the complications of these cysts, which include cholelithiasis, cholangitis, pancreatitis, intrahepatic abscess, biliary cirrhosis, portal hypertension and cancer, cysts should be excised surgically. This will involve bile duct resection with hepaticojejunostomy, as well as, partial hepatectomy in patients with intrahepatic involvement. In cases such as ours, with end stage liver disease, or those with Caroli disease, liver transplant is necessary.