As a multisystemic disorder with an unknown cause, Behcet’s disease requires further research in all aspects of pathogenic and clinical processes. The change in epidemiology possibly reflect the effects of environmental triggers, early intervention, and changing demographics. The etiopathology is better understood in recent years; however, immunogenetic components and triggering factors associated with an autoimmune, autoinflammatory, MHC-I-associated, and venous vascular inflammation need to be further investigated. The diagnosis depends on expert-defined phenotypic organ manifestations, but tools such as augmented skin reaction assays and measurement of femoral venous wall thickness seem to have a good predictive value. Clinical assessment of activity and damage requires better-validated multisystemic tools with biomarkers and imaging. Core outcome assessment with generic and organ-specific tools is studied by OMERACT Behcet’s study interest group for use in clinical trials. The current spectrum of immunomodulator and immunosuppressive treatments are effective for most patients; however, there is still an unmet need both for mild mucocutaneous disease and major organ manifestations.