A patient with Cantrell's syndrome is presented with ectopia cordis, ventricular septal defect and a left ventricular as well as a right ventricular diverticulum. One-stage correction was performed with resection of both diverticula. Closure of the ventricular septal defect was complicated due to exposure problems as a result of the malposition of the heart. Although the presentation of Cantrell's syndrome can be variable, one-stage repair is technically feasible and should be considered as the treatment of choice in this rare syndrome.