A retrospective study was undertaken to review demographic data, clinical presentation, outcomes, and long-term results of surgical repair of sinus of Valsalva aneurysms (SVAs). SVAs are a rare anomaly. Surgery is the treatment of choice. A retrospective review of an institutional database identified 86 patients who underwent SVA repair from 1956 to 2003. Follow-up ranged from 3 months to 40 years. The median age was 45 years (range 5 to 80). Approximately 44% of the patients had associated aortic regurgitation. Ruptures occurred in 34% of patients. The predominant fistula was from the right sinus of Valsalva to the right ventricle. Most (65%) were diagnosed by echocardiography, and the remaining (35%) were diagnosed on cardiac catheterization. All subjects underwent SVA repair. Seventy-two patients (84%) underwent other cardiac procedures at the time of aneurysm repair. Six patients (7%) died perioperatively, and the actuarial 10-year survival rate was 63%. In conclusion, echocardiography is the most frequently used diagnostic tool. The most common site of the aneurysm was the right coronary sinus. The concomitant surgical repair of associated ventricular septal defect, atrial septal defect, and the aortic valve is often required. Elective surgical repair can be performed with low risk.