A new syndrome of primary sclerosing cholangitis associated with fibrosis of the submandibular glands and the pancreas is described in a 43-year-old male. The sclerosing cholangitis was diagnosed at laparotomy because of cholestasis and the fibrosis of the submandibular glands and pancreas confirmed at microscopical investigation of biopsy specimens. The cholangitis responded well to treatment with a low dose of prednisolone (7.5--10 mg) and an endoscopic retrograde cholangiopancreaticographic examination 10 months after the operation revealed normal bile ducts.