Behçet's disease is a chronic multisystem disorder characterized by relapsing inflammation for which the underlying histopathology is an occlusive vasculitis. The disease has a high prevalence in the Mediterranean region, Far and Middle Eastern countries. It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. Uveitis is by far the most common ocular manifestation of Behçet's disease. It typically presents in the form of relapsing-remitting panuveitis with retinal vasculitis that may result in severe visual loss. Management of Behçet's uveitis relies on corticosteroid therapy, conventional immunosuppressive drugs and biological agents and requires a close collaboration between ophthalmologist and internist. A better understanding of auto-inflammatory process and of the role of cytokines implicated in the pathogenesis of Behçet's disease will enable researchers to develop more specific and effective therapy.