Background: Behcet's disease (BD) is an inflammatory multisystem disorder, characterized by recurrent oral and genital ulcers, erythema nodosum-like lesions, papulopustular lesions, uveitis, large vessel thrombosis as well as neurologic and gastrointestinal manifestations. Case Report: A 55-year-old man with BD was admitted to the emergency room with perianal abscess and necrosis. One day after abscess drainage and debridement the patient developed peritonitis, and sepsis ensued. Histopathologic examination of the skin overlaying the debridement area revealed vasculitis and fibrinoid necrosis. Emergency laporatomy revealed multiple 'punchedout' perforations with 0.5-1 cm in diameter over the whole small intestine concentrating in the terminal ileum. Debridement - primary repair and partial ileal resection - of ileostomy was done. The patient died due to sepsis and multiple organ failure on the first postoperative day. Histopathologic examination revealed nonspecific inflammatory cell infiltrates surrounding capillaries and venules, especially in the submucosa and serosa (vasculitis) with no evidence of granulomatous inflammation. Conclusion: Anal abscess or necrosis in a patients with BD may be a clue to the presence of intestinal BD with imminent perforation, representing the widespread vasculitis of the gut in BD.