The case of a mucin-producing intrahepatic cholangiocellular carcinoma in a 73 year-old-man is presented. A tumor originating in the right posterior inferior segment of the liver was found to be invading the right posterior and anterior bile ducts, and the hepatic hilus. Extensive superficial spread was observed in the entire posterior segmental bile duct extending to the hepatic hilus. Mucin produced and excreted by the tumor was retained in the common hepatic and common bile duct. The diagnosis in this case was suggested by percutaneous transhepatic aspiration of mucinous bile, and was confirmed by utilizing the techniques of ultrasonography, percutaneous transhepatic cholangiography, computed tomography and angiography. Curative surgery, which included right hepatic lobectomy with total caudate lobectomy and bile duct resection, was performed. Biliary continuity was maintained by left hepaticojejunostomy using a Roux-en-Y jejunal loop. The histological diagnosis was mucin-producing papillary adenocarcinoma originating in the right posterior inferior segment of the liver. Postoperative recovery was very good and the patient has now been enjoying a good active social life for the last 20 months with no signs of tumor recurrence. This case report discusses the unusual growth pattern of a mucin-producing intrahepatic cholangiocellular carcinoma involving the hepatic hilus, and suggests rational surgical treatment.

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