Aortic valve repair was performed in 61 patients having aortic regurgitation (AR) associated with ventricular septal defect (VSD). Preoperative regurgitation was classified according to the Sellers' classification and grade I was in 3 patients, grade II in 19, grade III in 34, and grade IV in 5 patients. Forty-six patients underwent only plication of aortic valve, and in 15 patients the commissuroplasty was added to reinforce the free edge of the cusp. Furthermore, aortoplasty (aortic annuloplasty) was performed in 11. Actuarial survival rate and freedom from reoperation rate were 91.6% and 87.2%, respectively at 10 years after the initial repair. The rate of freedom from deterioration for 22 patients showing grade I or II of AR before the initial operation was 86.4% at 1 year, 76.7% at 5 and 10 years, while the rate for 36 patients with grade III or more severe AR was 91.7% at 1 year, 77.4% at 5 years and 63.9% at 10 years. The rate at 10 years after operation between the two groups was different significantly (p < 0.05). Twenty among 22 patients having grade I or II of AR before operation were in NYHA class I, while 23 of 30 survivors with grade III or IV of AR were in class I, 6 in class II, and 1 in class III. Eight patients received reoperation and four of them underwent repeated conservative aortic valve repair, and one received aortic valve replacement at reoperation. Five of 7 survivors with reoperation improved in NYHA class I and 2 in class II. These results suggested that careful follow-up was required especially for the first 10 years after the initial operation in patients showing preoperative severe AR and receiving aortic repair.