Our experience with patients who underwent orthotopic liver transplantation for sclerosing cholangitis at the University of Pittsburgh Health Center between March 1980 and March 1988 is reported here. Ten patients had an associated cholangiocarcinoma. Six of these patients died of recurrent, disseminated cancer, usually before one year. One patient died of sepsis, while three are alive and apparently free of tumor four months to almost two years later. Pre-operative identification of a possible cholangiocarcinoma and complete resection are of crucial importance. Adjuvant therapy, especially pre-transplant radiation with a prophylactic purpose is still being evaluated.