Behçet's disease (BD) is a relapsing vasculitis in which orogenital ulceration is a prominent feature. The disease presents a tendency to thrombosis. The prevalence of venous or arterial thrombosis in BD reaches 40% in some series. Molecular markers of imbalance have been extensively studied in BD. The aim of this paper is to review current data on essential hemostatic parameters. The precise pathogenetic mechanism(s) underlying the prothrombotic state of BD is unknown. Thrombophilic factors could contribute to thrombosis in BD. Vasculitic endothelial injury may trigger or enhance the pathological hemostatic process.