Behçet's disease (BD) is a vasculitis in which the hallmark lesions are oral and often genital ulcers. Involvement of parenchymal central nervous system (neuro-Behçet's) is a serious complication commonly characterized by brainstem and/or basal ganglia lesions. To date, treatment of neuro-Behçet's remains largely empirical, and may not adequately control the disease. Serum tumor necrosis factor α (TNFα) levels are increased in active BD, suggesting a role for TNFα in disease pathogenesis. Clinically, significant improvement of various BD manifestations has been reported with TNFα blockade. However, evidence for the efficacy of TNFα blockers in the treatment of neuro-Behçet's is scant. We present 8 patients with neuro-Behçet's who responded favorably to infliximab therapy and review the relevant literature.