Adamantiades-Behget's disease is a systemic vasculitis of unclear etiology, with a worldwide distribution but more commonly seen in Mediterranean and Asian countries. The diagnosis of Adamantiades-Behget' s should be considered in patients presenting with orogenital ulcerations, skin disease, and inflammatory eye disease. Diagnosis can be difficult and requires a high index of suspicion, especially when not all diagnostic features are present. Treatment depends on the severity of disease manifestations, weighing the toxicity of medications with risk of adverse disease outcomes. Special attention should be paid to treatment of ocular disease, to prevent long-term morbidity from vision loss, and to identification of occult arterial aneurysms. Anti-TNF-a agents are proving to be effective therapies for disease and may reduce the need for cytotoxic therapy with Adamantiades-Behget's in the future.

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