Behçet syndrome (BS) is a multisystem vasculitis characterized by skin and mucosa lesions and musculoskeletal, ocular, gastrointestinal, neurological and major vessel involvement. It is seen mainly in the Mediterranean basin, Middle East and the Far East. The disease runs a more severe course among young males and the severity diminishes with age. This review describes the management of this disease, which should be individualized and varies according to site and gender.