Sclerosing Cholangitis is an uncommon disease which involves either all or part of the extrahepatic biliary duct system and, occassionally, affects the intrahepatic biliary radicles. The disease has also been called "obliterative cholangitis" and "stenosing cholangitis", in reference to a progressive thickening of the bile duct walls encroaching upon the lumen. Several authors have proposed that the temr "primary sclerosing cholangitis" be reserved for cases in which there arew no associated diseases, and that all other cases be classified as "secondary sclerosing cholangitis". Many rigid criterias have been established for the diagnosis of primary sclerosing cholangitis. Recently the authors experienced one case of primary sclerosing cholangitis which was coincided with rigid criterias and was confirmed by operation with histologic examination. We present our case and review the literatures.