OBJECTIVES: The aim of this study was to analyse the spectrum of echocardiographic findings in patients with cardiovascular involvement in Behçet's disease (BD) and followed up the post-operative complications. METHODS: We enrolled 26 BD patients who underwent first cardiac surgery in Anzhen Hospital, Beijing, China. Medical records and echocardiographic findings were retrospectively analysed. RESULTS: The 26 patients consisted of 4 women and 22 men. 22 (84.6%) of the patients were diagnosed with moderate/severe aortic regurgitation (AR). Some distinctive echocardiographic features with AR were observed, including prolapse of aortic cusps, vegetation-like mobile lesions, an echo-free space mimicking aortic root abscess and aortic aneurysm formation. 3 (11.5%) of the patients were diagnosed with isolated descending aortic aneurysm. 1(3.8%) of the patients was diagnosed with pulmonary artery aneurysm. BD was preoperatively diagnosed by clinicians in 20 patients. And 6 patients were diagnosed post-operatively by clinicians. In a total of 26 patients, post-operative complications occurred in 8 (30.7%) patients. The complications occurred in the 6 patients diagnosed post-operatively and 2 patients diagnosed pre-operatively. The post-operative complications of these patientsincluded aortic paravalvular leakage, coronary-graft anastmotic leakage and mitral paravalvular leakage. CONCLUSIONS: The most common echocardiographic feature of cardiovascular involvement in BD is severe aortic regurgitation with prolapse of aortic cusps, vegetation-like mobile lesions, an echo-free space mimicking aortic root abscess or aortic aneurysm formation. Accurate preoperative diagnosis of BD is beneficial to the choice of immunosuppressive therapy before and after surgery, which is likely to reduce postoperative complications especially for patients with severe lesions.