Two pulmonary vascular disorders can occur in liver disease and/or portal hypertension: the hepatopulmonary syndrome (HPS), which is characterized by intrapulmonary vascular dilatations, and portopulmonary hypertension (POPH), in which pulmonary vascular resistance is elevated. POPH and HPS are characterized by distinct pulmonary microvascular remodelling, which occurs at different anatomical sites of the pulmonary microcirculation. The exact pathophysiological mechanisms of these pulmonary vascular disorders are unknown. However, as HPS and POPH have been reported in patients with extrahepatic portal hypertension, the factor that determines their development must be portal hypertension. The clinical presentations are very different, with gas exchange impairment in HPS and hemodynamic failure in POPH. The severity of HPS seems to parallel the severity of liver failure, whereas no simple relationship has been identified between hepatic impairment and the severity of POPH. Resolution of HPS is common after liver transplantation, which has an uncertain effect in POPH.