A persistent left superior vena cava draining into the left atrium may produce a symptomatic right-to-left shunt. Although intra-atrial rerouting techniques, in patients with no connecting vein, have proved to be reliable and successful, in many cases the extracardiac repair is preferable. We report a case of a 5-month-old patient with a not connected left superior vena cava draining into the left atrium, associated with atrial septal defect and partial anomalous pulmonary venous connection. The correction has been achieved by rerouting the pulmonary venous return into the left atrium and by transposition of the left vena cava on the right appendage.