BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic liver disease, which usually affects young adults and is diagnosed by cholangiography. On a few occasions, the disease either starts in or exclusively involves the small intrahepatic bile ducts, referred to as small-duct PSC.

METHODS: A 31-year-old man presented with severe hematemesis secondary to liver cirrhosis. Over a course of 8 years, his liver decompensated and required an orthotopic liver transplantation. In this report we discuss his disease presentation, course of management, and the post-transplantation course of management, and review the morphologic diagnosis, and differential diagnosis of the disease with large-duct type and other diseases that involve small intrahepatic bile ducts.

RESULTS: The patient's explanted liver showed changes of PSC affecting only the small- and medium-sized bile ducts in addition to three incidental nodules of hepatocellular carcinoma.

CONCLUSIONS: Small-duct PSC has a substantially better prognosis than the large-duct type, with less chance of developing cirrhosis and an equal risk for developing hepatocellular carcinoma, but no increased risk for developing cholangiocarcinoma. Treatment seems to help relieve the symptoms but not necessarily improve survival. Liver transplantation remains the ultimate cure.