Behçet syndrome is a chronic, recurring, systemic disorder characterized by the histopathologic finding of nonspecific vasculitis in multiple organs. Behçet syndrome involves the gastrointestinal tract in 10-50% of patients; The main sites of involvement are the terminal ileum and cecum. In patients with Behçet syndrome, CT is advocated for early detection of complications as well as for exclusion of other abdominal pathologic conditions but there is no specific exam. The report of histology in conjunction with the clinical history and the presence of oral ulcers, uveitis and suspected cutaneous lesions suggests the diagnosis. The optimal medical treatment of Behçet syndrome has not yet been well established. In rare cases surgery must be required to control the disease. The authors report one case of Behcet Syndrome presenting with intestinal perforation.