Two pulmonary vascular disorders, considered mutually exclusive, may be present in candidates for orthotopic liver transplantation (OLT). On the one hand, hepatopulmonary syndrome (HPS), with a prevalence about 20% in end-stage liver disease, is characterized by pulmonary vascular dilatation and abnormal gas exchange. On the other hand, portopulmonary hypertension (POPH), a process defined by pulmonary hypertension associated with portal hypertension, is less common than HPS (4%). These entities have very distinct clinical implications; whereas HPS is clinically characterized by respiratory symptoms that evolve to severe hypoxemia, patients with POPH are commonly asymptomatic, frequently diagnosed in the setting of OLT, and the symptoms appear when there is hemodynamic compromise. The pathogenesis of both entities is a putative mechanism, the imbalance of vasoactive substances in pulmonary vessels. The role of OLT to reverse these vascular disorders is controversial, although complete resolution of HPS and, less frequently, POPH following OLT has been reported. The recognition that the presence of both HPS and POPH is an important cause of morbidity and mortality among recipients of OLT has resulted in a change in the policy to select OLT candidates. Accurate identification of patients with pulmonary vascular disorders associated with liver disease should be the first step in the management of OLT candidates. Because the determinants of the prognosis of OLT in the setting of these pulmonary vascular changes have not been well established, an accurate cardiopulmonary evaluation with careful assessment of pulmonary gas exchange (in HPS) and right ventricular function (in POPH) of potential OLT recipients is mandatory before the procedure.