A 60-years-old man was refferd to our hospital with elevation of hepatobiliary enzymes. The elevation of tumor markers was found. Abdominal contrast enhanced CT showed complete visceral inversion and tumorous lesion in the upper bile duct. ERCP findings showed the stricture of upper bile duct. Peroral cholangioscopy showed tumor extension from the central bile duct to the right hepatic duct. Cytology of the biliary stenosis was class V and diagnosised as cholangiocarcinoma. Because of the complete visceral inversion, the patient requested a surgery at a more specialized facility. Thus, right hepatectomy and caudate lobectomy and extrahepatic bile duct resection was performed. Histopathological finding was moderately differentiated tubular adenocarcinoma pT2aN1M0 stage IIIb.
