Behçet's syndrome is a multisystem disorder that causes orogenital ulceration, skin lesions and intraocular inflammation with uveitis and retinal vasculitis. A proportion of affected individuals also develop vascular and central nervous system manifestations, with significant morbidity and mortality. Although the aetiopathogenesis of Behçet's syndrome is poorly understood, the condition is considered to be driven, at least in part, by autoimmune mechanisms. Conventional therapy relies on available anti-inflammatory and immunomodulatory agents, and, in view of the paucity of controlled clinical trials, it is to a large extent empirical. Oral ulcers can often be treated by topical application of corticosteroids. In addition to corticosteroids, agents used to treat ocular inflammation and significant systemic manifestations include colchicine, thalidomide, azathioprine, mycophenolate mofetil, cyclosporin, tacrolimus, cyclophosphamide and chlorambucil. The response to these agents is variable and there is a distinct need for more effective rational treatment. Over the last decade, a number of open studies have produced promising results using recombinant interferon-alpha preparations. Evaluating, in a methodical manner, the other new biological agents that are becoming available for the treatment of inflammatory diseases offers great promise, not only for effective management but also for providing insights into aetiopathogenesis.