|Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic cholestatic disease characterized by an autoimmune-mediated destruction of small and medium-sized intrahepatic bile ducts. Originally the diagnosis of PBC was made in patients presenting with advanced symptomatic disease,inevitably indicating fatal outcomes. However, the development of antimitochondrial antibody (AMA) testing for early diagnosis as well as the introduction of ursodeoxycholic acid (UDCA) as the first-line therapy for PBC significantly changed the mortality of PBC. At present, patients at early stage have similar survival rates of an age and sex matched control population at least in the East Asia, as observed in the US and European countries. This drastic alteration of mortality is indeed the driving force for global agreement in the change of the nomenclature of PBC, from "cirrhosis" to "cholangitis". Meanwhile, there still remain several unmet needs regarding PBC; first, almost one-third of patients treated with UDCA exhibit incomplete responses and could progress to cirrhosis, and therefore we need to develop alternative treatment option for those with incomplete responses to UDCA. In this regard, obeticholic acid was recently approved in the US, but the efficacy and safety profiles are still unsatisfactory. Second, epidemiological data are still lacking in most of Asian countries/regions and need to be clarified. We definitely must reduce the burden of PBC in this area, with improved recognition and management of this disease.