Background: Erythropoietin-producing uterine myoma can cause various complications such as arterial or venous thrombosis and bleeding. Therefore, caution is required in the anesthetic management of affected patients. Case presentation: A 57-year-old female was suspected to have an erythropoietin-producing uterine myoma and was scheduled to undergo an abdominal total hysterectomy and bilateral salpingo-oophorectomy. Preoperative levels of hemoglobin and erythropoietin were 21.9 g/dl (normal 11.5-15 g/dl) and 23.2 IU/ml (normal 4.2-23.7 IU/ ml), respectively. Preoperative phlebotomy and isovolemic hemodilution were performed to prevent arterial and venous thrombosis, following previous evidence that a hemoglobin level < 16 g/dl reduces the occurrence of polycythemia vera-related complications. Fondaparinux 2.5 mg was subcutaneously injected once daily after the operation, resulting in a good perioperative course without major complications. Conclusion: Herein, we have described a successful perioperative management of a patient with erythropoietinproducing uterine myoma. Our findings in this case suggest that this combination of antithrombotic therapies can facilitate anesthetic management of patients with this disease.