Behçet disease is a multisystem inflammatory disorder, the cause of which remains unclear. Vasculitis is its predominant histopathological feature. It remains a source of significant morbidity in affected patients, many of whom become blind. Treatment of its various manifestations remains controversial today because of the paucity of randomized controlled trials and the absence of standardized outcome measures for this disease. The preferred treatment modalities combine different drugs, including topical therapies as well as systemic corticosteroids, NSAIDs, colchicine, and immunosuppressive and cytotoxic agents. The principal objectives are always relief of symptoms, control of inflammatory eye disease, suppression of systemic inflammation and vasculitis and prevention of recurrences and thus of irreversible damage. Although the prognosis of various manifestations of Behçet disease has improved, many patients still have refractory disease that requires treatment with combinations of various immunosuppressants, cytotoxic agents, and corticosteroids, which may lead to serious infections or secondary malignancy. Recent improvements in our understanding of the pathogenic mechanisms of Behçet disease, especially its molecular basis, have led to a new generation of potential treatments with improved side-effect profiles and more specific immune targeting. These include new immunosuppressants, biologic medicines, tolerizing agents and immunoablation techniques. Until randomized controlled studies with these agents are conducted, however, no final judgment about their usefulness is possible.