Portopulmonary hypertension (POPH) refers to the condition of pulmonary arterial hypertension (PAH) in association with portal hypertension. Among patients with portal hypertension, reported incidence rates of POPH range from 2 to 10% and long-term prognosis in cases of POPH is poor. Fundamental issues of management of POPH include identification of patients likely to benefit from liver transplantation (LTx) and treatment with specific pulmonary vasodilators before and after LTx. This review presents as an overview of the current knowledge on the epidemiology, diagnosis, treatment and prognosis of patients with portopulmonary hypertension.