Congenital diaphragmatic hernia is associated with a high mortality, especially when combined with other defects. The unusual repeated combination of the same congenital abnormalities by a number of patients and the fact that during embryonic life these organs develop from the mesoderm implies the existence of a specific syndrome. This article summarises the available clinical data and reports on three cases with Cantrell's syndrome, showing different pattern of congenital defects such as: 1) supraumbilical abdominal wall defects; 2) a defect of the lower sternum; 3) a deficiency of the anterior diaphragm; 4) a defect in the diaphragmatic pericardium; 5) congenital intracardiac defects.