Incomplete Cantrell's Pentalogy-A Case Report.

We present a case of an 11-year-old boy, who presented to us with a ventral hernia since birth. He was found to have the following: A large ventral midline defect starting from umbilicus to xiphisternum. Sternum was bifid at the lower end. Liver had three separate lobes. Extrahepatic biliary apparatus was normal and liver was ptotic. Diaphragm had an anterior midline defect. Diaphragmatic defect was in continuity with pericardial defect and heart could be seen directly pulsating through the skin. Despite modern surgical standards, Cantrell's syndrome represents a challenge to the surgeon because of the wide spectrum of anomalies, the severity of the abdominal and cardiac malformations, and the high mortality.