OBJECTIVE: To review Behçet's disease and to describe its clinical features in the head, neck and upper respiratory tract. METHOD: A literature review was undertaken, following a Medline search of publications over a 30-year period, and utilising the expert knowledge of one of the authors (RJM) with a specialist interest in Behçet's disease. RESULTS: Twenty-seven articles with ENT relevance were obtained. Otorhinolaryngological manifestations included symptoms and signs in the mouth, nose, sinus, larynx and ear. CONCLUSION: Behçet's disease is usually considered to be a condition affecting the oral cavity, eyes and genitals. This article shows that most patients will also exhibit other ENT symptoms, hearing loss in particular. Indeed, Behçet's disease may present with features other than the classic triad of symptoms. Raised awareness of the clinical features within the head and neck region will hopefully enable early diagnosis and treatment of this potentially serious condition.