We report a neonatal case of atresia of the coronary sinus ostium draining into a persistent left superior caval vein, associated with aortic arch hypoplasia, ventricular septal defect and mitro-aortic discontinuity. In this setting, left superior caval vein receives the coronary flow, which is directed into the innominate vein and the right atrium. It is mandatory to perform a precise anatomical definition of this unusual cardiac malformation, before repair of associated cardiac defects. Indeed, the interruption of coronary venous drainage could potentially lead to myocardial ischemia and necrosis.