Adamatiades-Behçet disease (BD) is a primary systemic vasculitis of unknown origin, that may involve blood vessels of all sizes. The clinical hall-mark of the disease are recurrent oral aphthous ulcers, painful, sharply margined and often associated with recurrent genital ulcers. A variety of skin lesions, such as erythema nodosum-like nodules and purpuric lesions are frequently observed. Less common manifestations of BD include thrombophlebitis, central nervous system and ocular inflammation which, however, may impact significant morbidity and mortality. The etiology is unknown, although genetic factors, infectious agents, oral and salivary microbioma, and immunologic mechanisms are implicated and studied. Topical and eventually systemic corticosteroids are the choice treatment.