Cantrell’s syndrome is a rarely encountered midline malformation involving the structures such as the sternum, abdominal wall, pericardium, diaphragm and the heart. Approximately one hundred cases have been reported up to date. The mortality due to ectopia cordis is substantial. In this study, we present a case of Cantrell’s syndrome with thoracoabdominal ectopia cordis along with its echocardiographic and computerized tomography (CT) angiography findings.