PBC is one of the main subgroups of chronic cholestatic liver disease. In 2015, the designation of PBC was changed from primary biliary cirrhosis to remove the "cirrhosis stigma" to more accurately reflect the disease and its natural course [1, 2]. PBC is a chronic cholestasis disease mediated by autoimmunity. It is characterized by a continuous autoimmune response leading to selective destruction of the small and middle bile ducts in the liver, intrahepatic cholestasis, which induces duct proliferation leading to bile duct cell death, liver fibrosis, cirrhosis, liver failure, and even hepatocellular carcinoma. The etiology and pathogenesis of PBC are still unclear. This paper, based on the factors leading to PBC immune disorders and the immune mechanism of PBC immune disorders, discusses the possible factors leading to immune disorders such as heredity, environment, drugs and intestinal flora disorders and the related immune pathogenesis.