Primary sclerosing cholangitis is a progressive liver disease for which orthotopic liver transplantation is the only curative procedure. Questions exist regarding the role of temporizing procedures and the timing of transplantation. During the past 4 yr, we have performed liver transplants in 177 adult recipients. Twenty-six patients (14.6%) with primary sclerosing cholangitis received 30 transplants including 12 men and 14 women. The recipients were examined for a number of preoperative and postoperative variables. The 4-yr actuarial survival in patients with primary sclerosing cholangitis after transplantation was 88%. Patients were segregated according to preoperative risk variables. Twenty patients were low and medium risk, with one death (95% survival). Three patients were high risk, with two deaths (33% survival). In conclusion, orthotopic liver transplantation is safe and effective therapy for primary sclerosing cholangitis. Early referral for transplantation is recommended to reduce the mortality associated with this procedure in those with advanced hepatic failure.