The hepatopulmonary syndrome is a disease entity seen in association with liver failure and other disease entities. It is a devastating consequence of liver failure that results in a significant morbidity for affected patients. Currently, there are no identified medications that ameliorate the symptoms of hypoxemia in this disease state. Recent research, however, has begun to unravel the pathobiology of the vascular dilations that arise in the lungs of patients with liver failure. In this article, a compendium of current knowledge is presented, as well as the contemporary methods for identifying and treating patients.