Primary biliary cirrhosis( PBC) is a chronic progressive cholestatic liver disease associated with immunity,with unknown etiology and pathogenesis. This article reviews the origin of its name,recent advances in research on PBC,and the prospect of treatment. Ursodeoxycholic acid is considered to be effective in improving cholestasis among PBC patients,but its naming and risk factors,as well as the response criteria and treatment,still need further research.