Behçet's disease is an inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Many abnormal echocardiographic findings have been previously reported in patients with Behçet's disease. Some of these; namely, aortic root dilation, aortic valve regurgitation, intracardiac thrombi, and pulmonary artery aneurysm may precede other manifestations of the disease and occasionally require surgical intervention for proper management. Compared to patients without Behçet's disease, management of these complications in patients with the disease is challenging and requires different treatment strategies. If diagnosis of Behçet's disease is missed prior to surgical intervention, higher incidence of surgical failure, recurrence, and even higher postoperative mortality may occur. In this review, the author discusses these major cardiac complications of Behçet's disease.