Pentalogy of Cantrell: a case report.

Pentalogy of Cantrell is a rare congenital anomaly characterised by deficiency of midline mesodermal structures with congenital heart disease. Here a six-year-old girl who underwent surgery was presented with a large abdominal swelling. She had a large supra-umbilical abdominal wall defect covered with coarse tissue through which the abdominal contents were herniating. The lower sternum and xiphoid were deficient. Collaboration between cardiac surgeons and plastic surgeons is essential for optimal treatment.