Cyclophosphamide, combined with high-dose glucocorticoids, remains the primary medical treatment for arterial and large vein involvement including vena cava, hepatic vein, mesenteric vein, and intracardiac thrombosis. Azathioprine is the initial agent for induction treatment of deep vein thrombosis in other sites and for maintenance treatment for all types of vascular involvement. Monoclonal tumor necrosis factor-alpha antibodies emerge as effective agents for the induction and maintenance treatment of vascular involvement of BS. Short-term anticoagulants may be added after ruling out aneurysms in patients with venous involvement. Embolization of the bleeding pulmonary aneurysms with coils or cyanoacrylate might be lifesaving in emergent conditions. Treatment with immunosuppressives before and after invasive interventions decreases the risk of post-operative complications and recurrences. Other biologic agents, such as tocilizumab and JAK inhibitors, require more data for the recommendation in the management of vascular involvement in BS.