During the period from 1985 to 1993 primary sclerosing cholangitis was diagnosed in 16 patients, five females and 11 males, mean age 43 years. 12 of the patients (75%) had inflammatory bowel disease. Among 262 patients with ulcerative colitis, cholangitis was found in ten, whereas the disease was diagnosed in only two (1.9%) of the 108 patients with Crohn's disease. In eight patients (50%) no progression of the cholangitis was observed. However, six patients (38%) died during the observation period, three 19% due to cholangiocarcinoma and one due to liver failure. Cholangiocarcinoma is frequently found in patients with primary sclerosing cholangitis. A mortality of 38% indicates a serious clinical course in many patients.