Background . The Ross operation, first performed in children in 1968, may be the ideal aortic valve replacement. Technical demands of the operation and two valves at risk have delayed acceptance. A review of our experience to assess midterm and long-term results with the Ross operation is presented. Methods . The records of 150 consecutive patients, aged 7 days to 21 years (median age, 12 years, 75% less than 15 years) were reviewed. Follow-up was complete within the last 12 months (median, 2.8 years; range, 1 month to 10 years). Echocardiographic assessment was available on 116 (71%) within 1 year of closure and in 136 (91%) within 2 years. Results . Survival was 97.3% at 8 years. Late autograft valve dysfunction required replacement in 2 and reoperation with restitution of autograft function in 6. Freedom from reoperation for autograft dysfunction is 90% ± 4% at 8 years. Freedom from reoperation for homograft obstruction is 94% ± 3% at 8 years. Pulmonary homograft dysfunction (gradient >40 mm Hg) was present in 4 additional patients. Freedom from reoperation on the homograft or a gradient of 40 mm Hg is 89% ± 4% at 8 years. All patients have a normal, active lifestyle, without anticoagulants for their aortic valve replacement. Conclusions . The Ross operation is the preferred operative replacement in children requiring aortic valve replacement.