Behcets syndrome (BS) is a multisystemic inflammatory disorder characterized by recurrent oral and genital ulcers and ophthalmic alterations, but also involving other systems, including joints, blood vessels, nervous, respiratory and gastrointestinal tracts. Its etiopathogenesis remains unknown, but epidemiologic data suggest an interaction among genetic, immunologic and infectious factors. BS has a worldwide distribution being most frequently seen in the Mediterranean area, Japan and Middle East. In Brazil there are no substantial data regarding its prevalence or incidence. The aim of the present study was to review the main epidemiologic data, clinical features, diagnostic criteria and current treatment of BS.