To describe clinical and epidemiological data of Behçet's disease (BD) in Brazil, we retrospectively reviewed records of all patients seen between 2006 and 2007 at the BD outpatient clinic of University of Sao Paulo. One hundred and six patients fulfilled the International Study Group for Behçet's Disease diagnostic criteria and they were included in this study. There was a female/male ratio of 2.2:1 and mean age at diagnosis was 31.9 +/- 9.2 years. In order of frequency, oral (100%) and genital ulcerations (92.5%), pseudofolliculitis (59.4%), erythema nodosum (49.1%), ocular lesions (47.2%), and musculoskeletal complaints (35.8%) were the most common manifestations. Blindness and major vessel involvement (18.2% vs. 5.5%, p = 0.038 and 27.3% vs. 9.6%, p = 0.019) were more frequent among male than female patients. We concluded that in Brazil, a South American country faraway from the Silk Route, BD follows the same pattern exhibited on its usual endemic area.