Pentalogy of Cantrell corrected by one-stage repair

Pentalogy of Cantrell (POC), is a rare congenital defect associated with five ventral midline abnormalities and high mortality. We report a 2,788 g female infant with partial repair of POC in the neonatal stage. This consisted of repairing the omphalocele, plicating the eventrated diaphragm and placing the heart into the thoracic cavity by fixing the pericardium to the ventral side of the diaphragm. She was well 6 months after surgery.