This is a report on a 36-year-old male patient presenting with a rare combination of ulcerative colitis and primary sclerosing cholangitis. The disease of the biliary tract was suspected on the basis of the endoscopic retrograde representation of the common bile duct, and serologically differentiated from a chronic destructive, non-supperative cholangitis on the basis of a lack of antimitochondrial antibodies. Subsequently, a hepaticojejunostomy was carried out to normalize the bile flow.