Primary biliary cirrhosis (PBC) remains one of the commoner indications for orthotopic liver replacement. The two main indications for transplantation are poor quality of life (because of the liver) or end-stage liver disease. A number of prognostic models have identified risk factors indicating poor prognosis, but in practice serum bilirubin greater than 150 mumol/L is used most commonly. Other indications for transplantation include progression of hepatopulmonary syndrome, increasing osteoporosis, evidence of malnutrition, and development of hepatocellular carcinoma. Postoperatively, patients do well. Recurrence of PBC remains controversial, but an increasing number of centers now report that a proportion of patients develop evidence of recurrent disease in the allograft. As yet PBC recurrence remains of little practical importance, although as survival increases beyond 10 years, this may become more relevant.