Abstract Primary sclerosing cholangitis is an uncommon condition of unknown etiology, presenting extrahepatic biliary obstruction due to a chronic inflammatory obliterative process. Criteria for diagnosis include progressive biliary obstruction due to a chronic inflammatory fibrous process, absence of biliary stones, absence of biliary surgery prior to onset of symptoms, and a follow-up period long enough to exclude sclerosing carcinoma of the biliary tree. Long-term prognosis is generally considered to be poor. Our patient was treated by resection of the hyperplastic fibrous inner wall making possible T tube drainage. Steroids and antibiotics were given, and the patient is alive and well three years later.