The pentalogy of Cantrell is a congenital anomaly characterized by variable association of a thoracoabdominal wall defect to defect of the lower sternum, diaphragm, pericardium and heart, the most representative is ectopia cordis. Cantrell in 1958 reported 5 cases with this anomaly. The prevalence of pentalogy of Cantrell has been estimated between 1 / 65, 000 to 1 in 200,000 live births. The etiology of this anomaly is not clear and is considered heterogeneous. The mechanism proposed embriopatologico suggests a flaw in the ventral migration of mesodermal cells. We report a case of pentalogy of Cantrell with all the classic features with prenatal diagnosis. It also suggests a hypothesis about the embriopatogenesis and integrated management of the mother and fetus.