Primary sclerosing cholangitis (PSC) represents an important indication for liver transplantation. Selection for and timing of liver transplantation is difficult due to the disease course and the frequent occurrence of hepatobiliary malignancies. Pretransplantation screening of malignancies is difficult, but brush cytology of the biliary ducts seems to represent a possibility for early detection of some cholangiocarcinomas. Patient and graft survivals following liver transplantation are good, with 1 year patient survival exceeding 90%. Survival is also satisfactory in patients with early detected or highly limited cholangiocarcinomas. Recurrent PSC represents a particular problem, and affects as many as 20 to 40% in a long-term perspective. Few predictors of recurrent disease have been identified; severe rejections and their treatment may be of importance.