Objective: The results of single-stage and 2-stage repair of coarctation of the aorta with ventricular septal defect have improved, but the optimal treatment strategy remains controversial. This study compares our results with these 2 approaches. Methods: We performed a retrospective analysis of 46 patients, 23 with single-stage repair and 23 with 2-stage repair, who underwent completed surgical treatment of coarctation of the aorta with a ventricular septal defect at the Children's Hospital of Michigan between March 1994 and June 2006. Results: The average number of operations in the single-stage group was 1.5 ± 0.6, and in the 2-stage group it was 2.2 ± 0.4 (P ≤ .0001). Postoperative complications were similar, except for the number of planned reoperations to perform delayed sternal closure in the single-stage operation (n = 7) compared with the 2-stage operation (n = 1, P = .023). The patient age in the single-stage group at the time of discharge (completed repair time) was a median of 39.0 days (range, 19-250 days) compared with a median of 113.0 days (range, 26-1614 days) in the 2-stage group after stage 2 (P ≤ .0001). Freedom from cardiac reintervention was 89.8% in the single-stage group versus 84.9% in the 2-stage group (P = .33). The hospital mortality was 4.4% (1 patient) in each group. The actuarial survival rate was 95.7% in the single-stage group versus 90.6% in the 2-stage group (P = .38). Conclusions: The advantages of single-stage over 2-stage repair of a ventricular septal defect with coarctation of the aorta include an earlier age at completion of repair, fewer operations, and fewer incisions. Postoperative complications and hospital mortality are similar. The one disadvantage of a single-stage repair was the increased need for delayed sternal closure compared with the 2-stage approach.