Hepatopulmonary syndrome is defined as the clinical triad of advanced liver disease, arterial deoxygenation and intrapulmonary vascular dilatation. Its pathogenesis is not completely understood. Excessive pulmonary nitric oxide production seems to be one of the factors that contribute to the intrapulmonary vascular dilatation. Other mediators such as endothelin-1 and the heme oxygenase-1/carbon monoxide system have recently been found to be important contributors. The major clinical manifestations are arterial hypoxemia, clubbed fingers and spider nevi. Orthodeoxia is the characteristic clinical feature. Contrast-enhanced echocardiography is the preferred screening test. 99mTechnetium macroaggregated albumin (Tc-99m MAA) lung perfusion scan can further specify the diagnosis of hepatopulmonary syndrome and quantify the magnitude of shunting. No clearly effective medical treatments have been found. Although liver transplantation seems feasible to reverse this situation, it is associated with increased postoperative morbidity and mortality. A preoperative arterial oxygen tension of 50 mmHg or less and Tc-99m MAA shunt fractions of 20% or more are strong predictors of postoperative mortality that can be used to stratify patients with better outcome.