Primary biliary cirrhosis (PBC) is a chronic liver disease characterized by an autoreactive T-lymphocyte mediated injury of interlobular bile ducts. However, the pathogenesis of this disease is not completely understood. In the light of current knowledge the development of PBC seems to be a result of environmental or genetic factors. Primary bile duct injury may be caused by infective, chemical or toxic factors. In genetically susceptible individuals this injury may lead to a T-cell mediated autoimmune response directed against bile duct epithelial cells. When immune mediated bile duct injury has been initiated, the disease leads to chronic cholestasis, fibrosis and hepatic cirrhosis. In this paper we discuss recent views on etiopathogenesis of PBC.