Pathological features of primary biliary cirrhosis (PBC) are reviewed. Immune-mediated, non-suppurative cholangitis is the initial lesion and is followed by the gradual and extensive destruction of bile ducts and development of chronic cholestasis. Simultaneously, necro-inflammatory activities of the hepatic parenchyma and limiting plates of milder form develop not infrequently. Eventually, liver fibrosis and cirrhosis develop. A new system applicable to needle liver biopsies in which staging is evaluated using a combination of three factors (fibrosis, cholestasis, and bile duct loss) and necro-inflammatory activities of the bile duct and hepatic parenchyma are graded, is proposed. The clinical and therapeutic evaluation of PBC using this system is warranted.