| Summary: | Behçet’s syndrome (BS) is a systemic vasculitis with a wide range of clinical presentations and disease courses. It may involve the mucosa, skin, joints, vessels, eyes, and nervous and gastrointestinal systems. These organ involvements may present alone or co-exist in the same patient. Three main clusters of commonly co-existing manifestations were recognised and are currently called disease phenotypes. There is a significant heterogeneity among patients regarding demographic features and clinical expression of the disease that hinders a standardised disease assessment and a generalised use of diagnostic criteria. Additionally, BS is not associated with pathognomonic laboratory or histopathology features; therefore, the diagnosis is mainly based on the clinical manifestations. The purpose of this narrative review of the literature is to provide a description of the most common or typical clinical features of BS, summarise the major phenotypes of BS, and address the diagnosis strategy of this syndrome.
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