Cantrell's syndrome is a rare combination of congenital midline thoracoabdominal defects and congenital heart disease. surgical repair can correct many of the anomalies. We report a neonate who presented with a short sternum, defective upper abdominal muscle, ectopic umbilicus, anterior diaphragmatic defect, ventricular septal defect and an intra-abdominal left ventricular diverticulum. Initially, the infant was treated with digoxin and diuretics for 5 months. Due to poor control of heart failure, one-stage surgical correction was performed with patch repair of the ventricular septal defect, ligation of left ventricular diverticulum and closure of the defects of the diaphragm and upper abdominal wall.