Congenital left ventricular diverticulum is a rare condition. When found, it is usually accompanied by other intracardiac malformations, so that again further examination is indicated. Furthermore, it is usually associated with thoracoabdominal wall defect, as seen in the spectrum of Cantrell's pentalogy, a congenital anomaly consisting of a lower sternal defect, diastasis recti, pericardial defect, anterior diaphragmatic hernia and cardiac malformation. This paper reports a case of Cantrell's Syndrome with left ventricular diverticulum. Successful total correction of ventricular diverticulum, ventricular defects and diastasi recti, was performed at two years of age. The clinical features, ambryology and surgical management of these defects are discussed.