ABSTRACT
A patient with mucin hypersecreting papillary adenocarcinoma of intrahepatic bile duct had jaundice and symptoms of cholangitis. Radiologic evaluations-ultrasonography and computerized tomographyrevealed passage disturbance of bile at the level of the distal common bile duct or ampulla of Vater. But, primary lesion was located at left intrahepatic duct proximal to the ductal dilatation. This peculiar phenomenon confused clinicians. A 59-year-old man was referred to our hospital for evaluation of recurrent cholangitis. Ultrasonogram, computerized tomogram and endoscopic retrograde cholangiography disclosed dilatation and amorphous filling defect extending from left intrahepatic bile duct to common bile duct suggesting choledochal cyst(type IVa). Preoperative endoscopic examination showed spillage of mucin through duodenal papilla. Abdominal exploration revealed mucin hypersecreting papillary adenocarcinoma of left intrahepatic duct and dilated distal common bile duct filled with tenacious mucin. Left hepatic lobectomy and Roux-en-Y hepaticojejunostomy were performed.