Liver transplantation (OLTx) has been investigated as a mode of therapy for malignancies. The efficacy of OLTx for the treatment of cholangiocarcinoma (CCA) has been somewhat controversial. We review the current literature on resective procedures for CCA and show that isolated intrahepatic CCA has a slightly better survival than extrahepatic disease. Results of OLTx for CCA are then reviewed, with specific attention to the experience at our center. Our results demonstrate that 1-year patient survival was 53%, and disease-free survival at 3 years was only 13%. Specific issues pertaining to the timing of OLTx in primary sclerosing cholangitis are then addressed. In summary, we believe that OLTx for known CCA results in a very poor patient survival. Those with incidental CCA found on explant histopathologic evaluation, without lymphatic involvement, may result in acceptable patient survival.