Hemophilic pseudotumor involving the spine is extremely uncommon and presents a challenging problem. Preoperative planning, angiography, intra and perioperative monitoring with factor VIII cover and postoperative care for hemophilic pseudotumor is vital. Recognition of the artery of Adamkiewicz in the thoracolumbar junction helps to avoid intraoperative neurological injury. We report the case of a 26-year-old male patient with hemophilia A, who presented with a massive pseudotumor involving the first lumbar vertebra and the left iliopsoas. Preoperative angiography revealed the artery of Adamkiewicz arising from the left first lumbar segmental artery. Excision of pseudotumor was successfully carried out with additional spinal stabilization. At 2 years followup, there was no recurrence and the patient was well stabilized with a satisfactory functional status. Surgical excision gives satisfactory outcome in such cases.