We describe a 35-year-old Chinese woman with Behçet disease complicated by recurrent gastrointestinal flares. During admission for acute lower abdominal pain, a computed tomographic scan of the abdomen showed thrombosis of the left ovarian vein. She was treated with increased immunosuppressant and oral anticoagulant. Although she was not compliant to oral anticoagulant with her international normalized ratio frequently subtherapeutic, her symptoms abated and the thrombosis resolved. There has been only 1 reported case of a patient with Behçet disease presenting with postpartum ovarian vein thrombosis and pulmonary embolism and no reported case of Behçet disease with ovarian vein thrombosis occurring outside pregnancy and the puerperium. Ovarian vein thrombosis is a rare cause of abdominal pain that should be considered in patients with Behçet disease.