Primary biliary cirrhosis is an autoimmune chronic cholestatic liver disease of unknown cause that usually affects middle-aged women. It is characterized by inflammatory destruction of the interlobular and septal bile ducts, which leads to chronic cholestasis and cirrhosis. The diagnosis should be considered in the setting of elevated alkaline phosphatase, immunoglobulin M level and the presence of antimitochondrial antibody in serum. Ursodeoxycholic acid is the only medication of proven benefit for these patients. Liver transplantation is only therapeutic option for patients who have end-stage disease.