Bile duct carcinoma is a rare complication of ulcerative colitis. In most of the cases it tends to occur together with primary sclerosing cholangitis predominantly in older males. The authors report a case of a 25 year old woman presenting with jaundice, 6 years after the diagnosis of colitis was made. The cause of the extreme extra- and intrahepatic bile duct dilation was revealed by endoscopic retrograde cholangiopancreatography and percutaneous transhepatic cholangiography showing polypoid tumor in the common bile duct. The histological result taken during the surgical exploration proved the diagnosis of adenocarcinoma. Radical pylorus preserving pancreato-duodenectomy was performed. Subsequently adjuvant chemotherapy was instituted according to the PAV protocol. This rare case proves, that a malignant bile duct tumor may develop in a young patient with ulcerative colitis without the presence of primary sclerosing cholangitis. The authors emphasise the connection between ulcerative colitis and bile duct carcinoma and the importance of the close follow-up of every patient with ulcerative colitis.