Background Behcet���s disease (BD) is systemic vasculitis, and a rare but significant cause of aortic regurgitation (AR). Serious postoperative complications such as valve detachment leading to poor prognosis are common. However, clinical features of this disease have not been fully characterized. Methods Between 1990 to 2007, 13 patients with AR due to BD underwent open-heart surgery in SNUH. Their clinical records were retrospectively analyzed. Results All patients were men, and their ages at 1st surgery were 38짹7 yrs. Mean follow-up (FU) duration was 59.3짹48.7 mo. Only 2 patients were preoperatively diagnosed as BD. Most patients underwent repeated surgeries (mean 2.3짹1.2), and 5 patients died. Among 4 patients whose preoperative echocardiography could be reviewed, 2 cases showed characteristic aneurysmal change and prolapse of noncoronary cusp. One of them was accompanied by echo-free space around aortic root. Other 2 cases showed valvular thickening and root dilatation, respectively. The clinical outcomes were strongly correlated with therapeutic modality. 11 patients finally underwent aortic root replacement (ARR) after repeated AVRs. Only 3 of them died. However, all 2 patients who had only AVRs died. 3 of 10 patients who took immunosuppressive therapy (IST) died. 2 patients who did not take IST all died. ARR and IST significantly associated with longer event (composite of redo-surgery and death) free period (for ARR or AVR, 51.7짹47.3 vs 11.8짹19.4 mo., p=0.02; for IST or not, 50.5짹50.5 vs 12.6짹15.9 mo., p=0.03). CRP (p=0.04) and ESR (p=0.07) during FU tended to be negatively correlated with event free period. Conclusions These results demonstrate that therapeutic modality (ARR&IST) have a significant impact on clinical outcomes of AR due to BD. The poor prognosis of this disease entity could be overcome through the early diagnosis using characteristic echocardiographic findings and physical examination and the selection of appropriate therapy.