Since MAYOR and CANTRELL described in 1953 and in 1958 the ectopia cordis associated to other four anomalies, few cases have been reported, and only a small part of them have survived. We report the case of a neonate who presented with the features of the pentalogy. On the 3rd day we found at operation the heart laying over the left hepatic lobe, no pericardium could be identified and diaphragm was absent at its anterior edge. We made an "isolating sac" with duramadre fixed to the anterior thoracic wall, to both sides and to the posterior diaphragmatic defect. The initial management of the ventral defect is described. At the age of two years we initiated the definitive treatment aiming to: 1. To protect the heart. 2. To correct the huge abdominal eventration following the closure of the omphalocele. We described in details the technic employed to cover the precordial defect, to reconstruct the diaphragm, and finally as we treated in three consecutives stages the huge abdominal eventration.