Abstract:A case of primary sclerosing cholangitis in which an endoscopic inspection through the fistula produced by percutaneous transhepatic biliary drainage was performed for the treatment of progressive jaundice and supprative cholangitis is presented. These treatments benefited the patient to some degree. After 3 years from the onset, the patient died of multi‐organ failure caused by disseminated intravascular coagulopathy. An autopsy revealed primary sclerosing cholangitis. No evidence of ductal stricture due to malignancy or underlying cholelithiasis was noted.This report may be the first which discusses the endoscopic findings of a biliary tract stricture in a patient with sclerosing cholangiti