The authors report the unusual case of a 35-year-old woman suffering from left leg numbness and radiculopathy due to multiple lesions in the central nervous system: one right parietal extracranial-intracranial lesion with invasion of the sensory cortex, and two intraspinal, intradural lesions compressing the spinal cord at T3-5 and S 1-4. Biopsy sampling of the extracranial part of the parietal lesion led to a diagnosis of myeloid sarcoma. Further examination revealed no evidence of leukemic disease or myeloproliferative disorder. An aggressive multimodal approach to treatment in this patient with a combination of chemotherapy, whole-body radiotherapy, and allogeneic bone marrow transplantation was started immediately. The patient experienced full neurological recovery and complete disappearance of all lesions. At the 7-year follow-up examination, there was no evidence of disease. To the authors' knowledge, this is the first report of a myeloid sarcoma with both intracranial and intraspinal manifestations in a patient without leukemia.