Cantrell's pentalogy may be defined as a failure of fusion of the midline from the sternum to the umbilicus. Thus, this malady consists of multiple anomalies of the sternum, heart, pericardium, diaphragm and umbilicus or anterior abdominal wall. According to the degrees of each anomaly, various operations can be planned as a one-stage operation or as a multi-stage operation and then palliative or corrective operations. The authors experienced a case of Cantrell's pentalogy that consisted of a bifid sternum, ventricular septal defect, atrial septal defect, ventricular diverticulum, dextrocardia, pericardial defect, anterior diaphragmatic defect and diastasis recti; all of these problems were corrected by a one-stage operation