For Behcet's disease patients with aortic root lesions, the disease often manifests as aortic valve regurgitation (AR). Following aortic valve replacement (AVR), many of these patients often suffer perivalvular leakage and valve dehiscence, requiring a second or third operation. In this study, we report the outcomes of 20 patients who underwent AVR to manage aortic root lesions caused by Behcet's disease, and the lessons we learned. From October 2013 to September 2019, a total of 50 patients with Behcet's disease underwent AVR at our institution. Among them, isolated AVR was performed in 15 preoperatively undiagnosed cases. The other 5 patients were preoperatively diagnosed and underwent modified AVR. All patients were contacted for a follow-up. Valve function was evaluated using echocardiography. The 15 preoperatively undiagnosed patients [age: 38.4±12.6 years (range, 24-63 years); 9 males, 6 females] underwent isolated AVR as their primary procedure. Echocardiography revealed that valve dehiscence occurred in 13 (86.7%) patients postoperatively after a mean interval of 10.8±8.4 months. These patients accepted a second operation, and 1 of them accepted a third operation because of a pseudoaneurysm of the distal anastomosis site. For the other 5 patients [age: 38.8±9.5 years (range, 27-55 years); 4 males, 1 female] who underwent modified AVR, neither AR nor prosthetic valve detachment were observed during the echocardiography follow-up, and none required a secondary operation. For behcet's disease patients with AR, there was a high rate of valve dehiscence after isolated AVR. When compared with traditional AVR, we found that modified AVR was the optimal choice for patients who received standardized preoperative treatment. 2021 Annals of Translational Medicine. All rights reserved.