Ectopia cordis associated with Cantrells pentalogy

Cantrells pentalogy with ectopia cordis is an extremely rare and lethal congenital anomaly, with a reported incidence of 1:100000 births in developed countries. We report a neonate who presented with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect. The neonate had respiratory distress with peripheral cyanosis and died because of acidosis and electrolyte imbalance before surgical intervention could be undertaken. We discuss the case and present a brief review of literature and of embryogenesis.