In this report we describe the surgical treatment of a 27-year-old patient with complete Cantrell's syndrome, i.e. multiple ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with tetralogy of Fallot. Resection of the diverticulum was combined with correction of the tetralogy of Fallot and thoracoabdominal defects. The postoperative period was uncomplicated. We have have found only one previous report describing resection of the diverticulum combined with correction of Fallot's tetralogy and thoracoabdominal defects in an adult. One-stage repair of these complex anomalies is technically feasible and should be the treatment of choice.