Abstract: Hepato-pulmonary syndrome (HPS) is a severe pulmonary complication of the end-stage liver disease,based on chronic liver disease and/or portal hypertension, pulmonary vascular dilatation, gas exchange disorder, and arterial oxygenation abnormality occur, resulting in hypoxemia and a series of pathophysiological changes and clinical manifestations.Animal experiments have revealed that pulmonary microvascular dilatation, angiogenesis, and accumulation of intravascular mononuclear macrophages were the main causes of gas exchange abnormality, but the specific mechanism is still unclear. HPS is lack of effective drug treatment, and liver transplantation is the only effective therapy. In this review, we focus on the recent advances in pathogenesis, clinical diagnosis and treatment of HPS.