Patients with Behçet's disease are at risk for multiple vessel-related complications including thromboses, stenoses, occlusions, and aneurysms. Surgical treatment of Angio-Behçet brings numerous challenges due to the peculiarities of the disease process and the high rate of complications. Recurrent vascular episodes are also quite common and Behçet patients require rigorous follow-up. In this review, we focus on the manifestations of Behçet's disease involving the venous system and the systemic arterial vasculitis focusing on the indications, workup, and techniques for surgical treatment. Several case studies from our own experience are presented together with supporting diagnostic imaging and the decision process whether to intervene is discussed. Although open surgery remains a valid option, new endovascular techniques are rapidly advancing and offer excellent results with important decrease in morbidity and mortality even in highly compromised patients.