Primary biliary cirrhosis (PBC) is a disease characterized by inflammatory destruction of the small bile ducts within the liver. PBC eventually leads to cirrhosis of the liver. The cause of PBC is unknown, but because of the presence of autoantibodies, it is generally thought to be an autoimmune disease. Other causes, such as infectious agents, have not been completely excluded. In this issue, the guest editor has expert authors present information on important topics such as epidemiology of PBC, pathogenesis of PBC, medical therapies, and liver transplantation in PBC.