Intramedullary myxopapillary ependymomas are rare spinal cord tumors primarily affecting young adults. Grade 2 tumors are associated with a higher proliferative index and potentially more aggressive behavior compared to grade 1 tumors. We present a case of a 30-year-old male who presented with a three-month history of progressive unilateral lower back pain that was refractory to analgesics. Neurological examination revealed bilateral lower limb weakness and sensory impairments in the L2 region. MRI confirmed a well-defined, enhancing intramedullary lesion at the L2 level, causing cord enlargement and edema. Diagnosis of grade 2 intramedullary myxopapillary ependymoma was made. Complete surgical resection was performed, confirming a grade 2 myxopapillary ependymoma. Postoperatively, the patient demonstrated significant improvement in lower limb function and sensation, with no tumor recurrence during long-term follow-up. Rehabilitation therapy was initiated, while close monitoring for complications and tumor progression was maintained. This case explores the etiology and features of intramedullary myxopapillary ependymomas and underscores the importance of early recognition, accurate diagnosis, and aggressive surgical management.