We describe a 54-year-old asymptomatic male with carcinoma of the hepatic hilus. Elevated serum transaminases were detected during the annual medical examination. The diagnosis was confirmed by ultrasonography (US), computed tomography (CT), percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP). Curative surgery, which included right hepatic lobectomy with total caudate lobectomy, was performed. The resected specimen revealed a localized tumor in the right anterosuperior dorsal intrahepatic bile duct branch. The histological diagnosis was moderately differentiated tubular adenocarcinoma. The patient's postoperative recovery was smooth and he has remained in good health for 15 months after surgery without any signs of recurrence. This case report discusses the early diagnosis and rational surgical treatment for carcinoma of the hepatic hilus.

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