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Treatment of <jats:styled-content style=\"fixed-case\">BD<\/jats:styled-content> changes according to organ involvement, gender and age of the patient with no golden standard therapeutic regimen. Vascular involvement is observed in up to 40% of the patients with <jats:styled-content style=\"fixed-case\">BD<\/jats:styled-content>, especially in young males and is one of the major causes of mortality and morbidity. Glucocorticoids, azathioprine and cyclophosphamide are still recommended as the first\u2010line treatments in vascular <jats:styled-content style=\"fixed-case\">BD<\/jats:styled-content>. However, increasing data with the tumor necrosis factor inhibitors suggest that these agents may also be acceptable options for the management of refractory vascular <jats:styled-content style=\"fixed-case\">BD<\/jats:styled-content> in daily practice. Anticoagulant usage for vascular <jats:styled-content style=\"fixed-case\">BD<\/jats:styled-content> is also still controversial with limited data coming from retrospective studies. 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