Vascular involvement is a lethal complication in Behçet disease. It is often refractory to conventional therapy such as steroids and immunosuppressants in addition to anticoagulants. We describe here successful treatment with the anti-tumor necrosis factor-alpha (anti-TNF-α) antibody, infliximab, in a patient with Behçet disease presenting with deep vein thrombosis. A 60-year-old man with Behçet disease complained of edema and pain in the lower extremities. Computed tomography revealed a thrombosis extending from the popliteal vein to the inferior vena cava at the level of the renal vein and which recurred despite combination therapy of steroid and immunosuppressants such as cyclosporine, azathioprine, and methotrexate. The patient was then administered infliximab (5 mg/kg) in weeks 0 and 2 and every 4 weeks thereafter. Clinical and laboratory findings improved after the infliximab therapy. Computed tomography of the abdomen and lower extremities showed a reduction of the thrombosis. No severe adverse events occurred during the clinical course. Although further studies are needed to confirm the efficacy and safety of its use, anti-TNF-α antibody may be worth considering as treatment for refractory venous thrombosis in patients with Behçet disease.