Nine patients with sclerosing cholangitis were operated upon during the past seven years. Although all were icteric, the bilirubin level was only moderately elevated, while the alkaline phosphatase level was frequently markedly elevated. Large nodes along the common bile duct and nonspecific inflammation and fibrosis in periductal areas were found. It is postulated that the changes are due to bacteria entering the portal circulation from the bowel. An autoimmune origin seems unlikely, particularly as one patient developed the disease while receiving steroid and immunosuppressive therapy. Operation was necessary to diagnose the disease and decompress the biliary tract. Despite T-tube drainage and steroid therapy, six of the patients have died. In them, even when early palliation was obtained, the disease progressed, leading to secondary biliary cirrhosis with eventual liver failure, bleeding varices, coma, and death.