Primary sclerosing cholangitis (PSC) is a rare disease entity. The medical therapy for PSC has not been reasonably beneficial. Thus liver transplantation is known to be the ultimate therapy. Because liver transplantation for PSC has been performed rarely in Korea, we report a case of liver transplantation for PSC with a review of the literature. A 35-year-old male was admitted to our hospital with recurrent jaundice for seven years. ERCP showed multiple strictures of intrahepatic duct and an irregularity of the extrahepatic duct wall. Despite medication and endoscopic treatment, liver functions did not improve and clinical status got worsened. Thus liver transplantation was performed for the correction of hepatic failure. Two months after transplantation, liver functions and general weakness gradually improved and now, one year after liver transplantation, the patient is in normal life.