Myeloid sarcoma (MS) is an uncommon extramedullary tumor comprising immature myeloid cells, primarily associated with acute myeloid leukemia (AML). Spinal involvement is observed in fewer than 1% of cases. We present an unusual case of extramedullary MS in a patient with AML. A 23-year-old woman with a history of AML presented with neurological symptoms, including limb pain, weakness, and numbness. Imaging revealed an intramedullary enhancing mass at the cervical level. Partial laminectomy and microsurgical resection were performed, followed by laminoplasty. Immunohistochemistry supported the diagnosis, confirming MS. The patient was subsequently transferred to the hematologic department and received tailored treatment. The diverse clinical manifestations of spinal MS pose diagnostic challenges. In this case, an atypical intramedullary location led to incorrect initial diagnostic impressions. Comprehensive evaluations, including repeated biopsies and immunohistochemistry, were crucial for an accurate diagnosis. Treatment strategies vary based on systemic involvement, and prompt diagnosis is important for favorable outcomes. MS, especially in the spine, presents diagnostic and therapeutic complexities. Timely recognition, an accurate diagnosis through comprehensive evaluations, and tailored treatment strategies are crucial for positive outcomes in this rare hematologic entity.