Primary sclerosing cholangitis is characterized by inflammation and fibrotic strictures of the intra- and extrahepatic bile ducts. The diagnosis is confirmed by cholangiography, either endoscopic or percutaneous. An association between primary sclerosing cholangitis and chronic ulcerative colitis has been recognized by physicians for years. To date, there is no known cure for this disease. Several drugs are currently being utilized for symptomatic relief. Therapeutic radiologic and endoscopic procedures are performed to relieve obstruction of the bile ducts and reduce the back pressure on the liver. Surgical procedures are also designed to relieve biliary obstruction and to protect hepatic function. Recently, liver transplantation has become an option for some patients.