In a 40-year-old patient unexplained recurrent attacks of epigastric colic with transient cholestatic icterus occurred over a 9-year period. When the patient was again hospitalised because of progressive pain-free icterus associated with mild pruritus (alkaline phosphatase 900 U/l, direct bilirubin 305 mumol/l, GOT 187 U/l, GPT 103 U/l) sonography revealed liver enlargement to 17 cm, extended intrahepatic bile ducts and an echodense area of about 1 cm size in the region of the bifurcation of the common hepatic duct. Fine-needle puncture did not yield clear cytological findings. Endoscopic retrograde cholangiopancreatography pointed to sclerosing cholangitis. This diagnosis was confirmed by liver punch biopsy. Since the patient did not agree to a liver transplantation, he was treated with 450 mg ursodeoxycholic acid twice daily, resulting in marked reduction of the liver parameters until severe cholangiosepsis and acute renal failure occurred about 4 months later. The septic condition and its complications could not be managed despite thorough intensive-care measures so that a liver transplant had to be performed after all. Histology of the explantate revealed a cholangiocarcinoma in the region of the bifurcation of the common hepatic duct. At first the patient's condition improved markedly but one and half months later the transplant was rejected and the patient died.