Considerable discrepancies exist in the literature with respect to plasma total homocysteine (tHcy) levels in Behçets disease (BD). The aim of this study was to evaluate tHcy concentrations in these patients. Thirty-two patients with BD and 20 age- and body mass index-matched healthy volunteers were enrolled. Plasma tHcy concentrations were significantly higher, while vitamin B₁₂ and folate levels were significantly lower in patients with thrombosis and eye involvement than those without. C-reactive protein levels also correlated significantly in a negative manner with vitamin B₁₂ and folate but positively with tHcy. In conclusion, increased use or accelerated catabolism of folate and vitamin B₁₂ due to chronic inflammation and moderately increased tHcy concentrations related with deficiency of these cofactors, and immunosuppressive drug administration might be potential threats of vascular disease in BD.