Objective: The optimal surgical strategy for the repair of coarctation of aorta with ventricular septal defect(VSD) is controversial. This study was undertaken to determine outcome of single stage total correction of coarctation of aorta with VSD in the single institution. Method: Retrospective review of patients undergoing single stage total correction between January 1, 1995 and December 31, 2007. Results: Single stage total correction was performed in 72 patients (43 males) at a median age of 28 days (range: 3~188 days) and median weight of 3.7 kg (range: 2.16~5.6 kg). The VSD was perimembranous in 42, subarterial in 26 and muscular in 4 patients. Arch hypoplasia was present in 63 patients. Repairs were performed via a median sternotomy. Total circulatory arrest was performed in 22 patients and selective antegrade cerebral perfusion was performed in 49 patients. There was no operative death and one late death at a median follow-up of 38.2 months (range;0~158.1 months). Left main bronchus compression which needed aortopexy occurred in 1 patient. Postoperative subaortic stenosis occurred in 4 patients and surgical repair were performed in 2 patients. Recoarctation of aorta occurred in 2 patients and intervention with balloon dilatation was performed in 1 patient. Conclusion:. The advantages of single stage total correction over 2-stage repair of coarctation of the aorta with VSD include an earlier age at completion of repair and it can be performed with low mortality and morbidity as in this study.