Primary sclerosing cholangitis is a rare affection. Two characteristic cases are described. A total of 80 cases have been reported in the published literature, and lesions are found varying from lacalized to diffuse forms. The affection is often associated with other disorders, especially hemorrhagic ulcerative rectocolitis, suggesting an auto-immune pathogenicity. Treatment is mainly surgical and consists, in the localized forms, of complete excision whenever possible and a by-pass operation. The diffuse forms, which are difficult to treat by surgicall methods, have a poorer prognosis and usually progress towards secondary biliary cirrhosis.