Behçet's disease (BD) is now mentioned in the latest Chapel Hill nomenclature of vasculitides and is classified under the variable vessel vasculitides (VVV). Pathogenetically, a new classification among the so-called mixed-pattern diseases between classical polygenic autoinflammatory disorders and autoimmune diseases is being discussed. The genetic association with HLA-B51 is undisputed and an association with HLA-A26 as well as with polymorphisms in the IL-10 and IL23R-IL12RB2 genes have recently been described. Increasingly, a participation of IL-17 in the pathogenesis of BD is assumed. Therapeutically, the EULAR recommendations are still applicable. Interferon-alpha can be discontinued for severe ocular BD in remission without further relapses. Infliximab can be switched to adalimumab effectively and recent case series show an efficacy of IL-1 antagonists, tocilizumab and rituximab for BD.