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Introduction: Behcet’s Disease (BD) is classified among vasculitides. The aim of this review was to put together different known reports in order to help the reader to better understand the disease, to avoid the frequent misdiagnosis, and to decide the best treatment.

Areas covered: a) Epidemiology: BD is rare, and is seen along the Silk Road, from 20 to 420/100,000 in Turkey and 80/100,000 in Iran, to 0.64/100,000 in the UK. b) Clinical manifestations: oral aphthosis is seen in more than 95% of patients, genital aphthosis (60–90%), skin (pseudofolliculitis/erythema nodosum, 40–90%), eyes (uveitis/retinal vasculitis, 45–90%), gastrointestinal (diarrhea/hemorrhage/perforation/pain, 4–38%), vascular (venous/arterial thrombosis, aneurysm, 2.2–50%), neurological (all kinds, especially meningo-encephalitis, 2.3–38.5%), and articular (arthralgia/arthritis/ankylosing spondylitis, 11.6–93%). c) Pathergy test is positive in …