We report 2 cases of extrathoracic heart (ectopia cordis) operated on a few hours after birth at Laennec Hospital, Paris. The first patient had an associated diastasis of the rectus muscles. The second one, in whom diagnosis was made by ultrasound during fetal life, had a normal abdominal wall. In both cases it was possible to accommodate the heart in the left pleural cavity after a wide dissection of the posterior mediastinum. Both babies, however, died soon after the operation. Double outlet right ventricle was found in both. We have reviewed those cases previously described and discuss the importance of prenatal echocardiographic diagnosis in this rare condition.