Background: Behçet’s disease (BD) is a variable vessel vasculities that involves several organs and systems, causing ulcers on the oral, genital and intestinal mucosa, skin lesions that are most commonly in the form of papules, pustules or nodules, arthritis, uveitis, central nervous system lesions, venous and arterial thrombosis and arterial aneurysms. The exact pathogenic picture of BD is far from being clear. Whether it should be classified as an autoimmune or an auto inflammatory condition had been extensively debated. Early theories pointed to an autoimmune process triggered by an infectious or environmental agent in genetically predisposed individuals. Behçet disease, a chronic recurrent systemic inflammatory vascular disease, may affect blood vessels of any type and size. The disease can present with variable clinical manifestations and the most commonly involved systems are oral, ocular, cutaneous and urogenital. The diagnosis of BD is mainly based on clinical manifestations after ruling out other potential causes. There is no specific laboratory, histopathological, or genetic findings for the diagnosis of BD. Furthermore, there is a large geographical variation both in the disease prevalence and the disease manifestations. Therefore, the diagnosis of BD may be difficult in patients presenting with only major organ involvement such as posterior uveitis, neurologic, vascular, and gastrointestinal manifestations. The emergence of other disease manifestations aiding the definite diagnosis of BD can take months and even years in this group of patients. The disease can also remain limited in some patients, which causes diagnostic difficulty. BD typically runs in a relapsing and remitting course, and the goal of treatment is to promptly suppress inflammatory exacerbations and recurrences to prevent irreversible organ damage, A coordinated multidisciplinary approach is necessary for optimal care, treatment choices should be individualized based on age, sex, type, and severity of organ involvement as well as patient preferences. Ocular, vascular, neurological, and gastrointestinal involvement may be associated with a poor prognosis. Given the clinical heterogeneity of BD, the therapeutic approach is highly variable and is guided by the predominant disease manifestation