We report the case of a patient with liver cirrhosis who was admitted to the emergency room for rapid occurrence of dyspnea and severe hypoxemia at rest. Lung CT-scan and echocardiography did not disclose any right-to-left shunt and right-sided heart catheterization evidenced major precapillary pulmonary hypertension. The present feature supports the hypothesis that the pulmonary complications of cirrhosis, the hepatopulmonary syndrome and the portopulmonary hypertension, which are usually considered as mutually exclusive, may coexist. In such circumstances, the right failing heart is the major determinant to the immediate prognosis.