Behçet's disease (BD) is a systemic inflammatory disease of unknown etiology. The disease is strongly associated with the human leukocyte antigen (HLA) B51. BD has a chronic course with periodic exacerbations and progressive deterioration. There are no specific diagnostic laboratory tests, although recurrent oral ulceration is an obligatory manifestation for diagnosis. The treatment, which includes local, systemic, or surgical therapies, is based on the severity of the illness; the most appropriate management requires a multidisciplinary approach. This paper summarizes all aspects of BD with particular emphasis on the latest immunologic and treatment aspects.