Myeloid sarcoma is a rare extramedullary malignant tumor, which is often accompanied by the development of systemic myeloid disease at various sites. The involvement of the central nervous system is uncommon and spinal cord compression is particularly rare. In November 2012, a 27-year-old male presented with a paroxysmal headache, accompanied by nausea and vomiting, which had persisted for one year, and eyesight deterioration that had been apparent for five months. Magnetic resonance imaging (MRI) indicated a space-occupying disorder, a craniotomy to resect the brain tumor was undertaken, the pathological diagnosis of which was myeloid sarcoma. Two months after receiving 40 Gy of radiotherapy, the patient experienced numbness of the right thigh. MRI of the lumbar spinal canal revealed a mass involved both inside and outside the lumbar spinal canal. Pathological examination of the mass following resection also indicated myeloid sarcoma. Immunohistochemical analysis was positive for the ETO fusion gene in the bone marrow. Following six cycles of chemotherapy treatment, the patient achieved complete remission. At present, the patient is stable and is attending follow-up examinations regularly.