Behçet's disease (BD) is a chronic refractory multi-system autoimmune disorder with a strong genetic component. Like many other human complex diseases, multiple genes with polymorphisms have been associated with BD. These genes encode proteins involved mainly in immune regulation and inflammation and some in transcriptional activation and post-translational modification. Understanding the genetic association of these genes with BD may provide insight into the pathogenesis and for development of new, targeted therapies for this human complex disease.