Primary sclerosing cholangitis is an uncommon condition, generally considered to carry a poor prognosis. Based on the distribution of the biliary strictures, treatment groups can be defined. Those patients with localized hilar or predominantly extrahepatic strictures are most likely to be suitable for biliary-enteric bypass and the use of surgical techniques developed for the management of high bile duct strictures are allowing improvement in the results of surgery in this form of the disease. After 3 months to 3 years follow-up (median 16 months) nine of 12 patients treated by surgical biliary decompression are asymptomatic.