Hepatopulmonary syndrome is part of the spectrum of pulmonary vascular disorders seen in advanced liver disease. The pathophysiology of these entities likely is dependent on the degree of pulmonary vasoconstriction or vasodilation that occurs. Our understanding of hepatopulmonary syndrome has helped further our knowledge of the interaction of the liver and the lung. Advances in the management of this disorder, especially liver transplantation, finally have allowed us to offer some hope to patients with this disease.