Background Sinus of Valsalva aneurysm is a rare cardiac anomaly, and the difference between Asian and Western countries in its occurrence is not well established. This study was designed to investigate the difference between Asian and Western patients. Methods Between September 1988 and February 2006, 83 patients with sinus of Valsalva aneurysm underwent surgical repair in our institute. The aneurysms originated from the right and noncoronary sinus in 74 and 9, respectively, and ruptured into the right ventricle in 52 patients, the right atrium in 30, and the left ventricle in 1. Ventricular septal defect (n = 38), aortic regurgitation (n = 21), and bicuspid aortic valve (n = 4) were the common coexisting anomalies. To compare the differences between Asian and Western patients in sinus of Valsalva aneurysm, 1049 cases (654 Asian patients versus 395 Western) were collected from the literature. Results Sixty-six patients were followed up for 9.6 ± 3.8 years. The cardiac function of 15 patients with aortic regurgitation was worse than that of those with no aortic regurgitation ( p < 0.05). There was no difference between the direct closure and the patch closure ( p > 0.05). Analysis of all collected cases revealed that aneurysm of the sinus of Valsalva in Asian patients compared with Western series is characterized by a higher incidence, more aneurysms originating from the right coronary sinus (85.8% versus 67.9%), more aneurysm rupture into the right ventricle (72.5% versus 60%), a higher incidence of association with ventricular septal defect (52.4% versus 37.5%), and lower incidence of association with bicuspid aortic valve (0.6% versus 7.8%). However, both Asian and Western patient series have similar incidence of combination with aortic regurgitation (33.6% versus 32.7%). Conclusions Long-term results of ruptured sinus of Valsalva aneurysm are associated with preoperative aortic regurgitation. The difference between Asian and Western patients with ruptured aneurysm of the sinus of Valsalva is significant.