Venous complications in Behcet disease (BD) affect nearly 40% of patients. We describe a woman with BD who presented with a massive bilateral pulmonary embolism 30 hours after total hip arthroplasty. The patient underwent surgical thrombectomy and venoarterial extracorporeal membrane oxygenation. Methotrexate was withheld 15 days before surgery and restarted on postoperative day 2. Immunosuppressive therapy, such as methotrexate, appears to be the most effective prophylactic treatment for venous thrombosis in patients with BD, whereas prophylaxis with low-molecular-weight heparin may be ineffective, as was the situation in our patient. Therefore, continuation of methotrexate perioperatively should be considered in patients with BD. Systematically performing preoperative deep vein thrombosis screening should be also considered. The patient also had diabetes mellitus treated with metformin and sitagliptin preoperatively.