Portopulmonary Hypertension (PoPH) is a type of pulmonary arterial hypertension that uniquely afflicting patients with underlying liver disease and portal hypertension. The mainstay of treatment centers around the use of targeted pulmonary vasodilator therapy and evaluation for liver transplantation. This review article focuses on the role of targeted pulmonary vasodilator therapy and liver transplantation in the management of PoPH, with special emphasis on the expected hemodynamic and clinical effects of treatment. The relationship between PoPH and Hepatopulmonary Syndrome (HPS), another type of pulmonary vascular disease due to liver disease, is also reviewed.