We herein report a patient with strictures of the hepatic hilar and intrapancreatic bile duct associated with autoimmune pancreatitis, who responded well to corticosteroid therapy. Although this bile duct lesion resembled primary sclerosing cholangitis (PSC) morphologically,its dramatic response to corticosteroid therapy seems to indicate that it is a different entity. A review of the literature revealed 13 patients with multiple biliary strictures and chronic pancreatitis successfully treated with corticosteroid therapy. From the clinical point of view, knowledge of the existence of a patient group with autoimmune pancreatitis with morphology similar to that of PSC and which responds well to corticosteroid therapy is important.