Portopulmonary venous anastomosis are a very rare complication of chronic liver diseases. We report on a patient with a cryptogenic liver cirrhosis and thrombosis of the portal vein who underwent antibiotic treatment because of recurrent pneumonias several times. Although treated successfully a pulmonal infiltrate persisted in further radiologic controls. By means of a velocity-encoded MRI a portopulmonary shunt of 30% of the cardiac output was assured. An operative correction with a distal splenorenal shunt was performed successfully. Former reports of portopulmonary anastomoses complicating chronic liver disease never were hemodynamically relevant. In the presented case, a portopulmonary anastomosis lead to recurrent pneumonias and a restrictive ventilatory disorder.