Primary sclerosing cholangitis presenting as a localized stricture affecting a segment of the extrahepatic biliary tree is seldom found. This article describes the case of a 56-year-old male admitted with obstructive jaundice, in whom endoscopic retrograde cholangiography showed a localized and smooth stricture at the common hepatic duct, initially diagnosed as cholangiocarcinoma. However, the histological examination of the resected stricture, common bile duct and gallbladder, and a liver biopsy performed 3.5 years later were consistent with primary sclerosing cholangitis, affecting both the extrahepatic and intrahepatic biliary tree.