Behçet's syndrome (BS) is a systemic vasculitis that affects vessels of all calibers. It has several defining characteristics, such as its tendency for remission over time and a typical geographical distribution. Clinically, the association of venous thrombosis with arterial aneurysms, inflammatory parenchymal brain involvement, the classic pattern of posterior uveitis with retinal vasculitis, and the well-known triad of bipolar ulcers and erythema nodosum are distinctive features of this condition. Despite some advances in the pathogenesis of BS and the use of biological drugs that have improved prognosis, certain aspects remain controversial, such as the role of the pathergy test or the determination of HLA-B*51 in the diagnosis of the disease, or the actual value of anticoagulation in patients with BS and vascular thrombosis.