Patient: Male, 16-year-old Final Diagnosis: Hemorrhagic myxopapillary ependymoma • myxopapillary ependymoma Symptoms: Gait abnormality • pain in lumbar region Medication: — Clinical Procedure: Resection of intraspinal mass Specialty: Neurosurgery • Radiology Objective: Unusual clinical course Background: Spinal myxopapillary ependymoma (MPE) is a slow-growing tumor arising from ependymal cells of the central nervous system. MPE rarely presents with acute neurological compromise and most commonly occur in the filum terminale or conus medullaris region. To date, only a few cases have been reported of patients presenting acutely because of hemorrhagic MPE. Case Report: A 16-year-old boy without previous medical problems presented with a sudden onset of severe pain in the low back radiating to the thighs. He could not walk owing to the severity of the pain. Neurological examination revealed an unsteady gait, but the rest of the motor and sensory examination was normal. Lumbosacral spine magnetic resonance imaging revealed an intradural hemorrhagic mass extending from L5 to S2. The encapsulated hemorrhagic tumor was resected, and the pathology was consistent with MPE grade I. The patient made a significant recovery postoperatively. It is extremely rare for MPE to present with spontaneous hemorrhage in the lumbosacral region. Prompt diagnosis and management led to a favorable outcome. This case report is intended to highlight the atypical presentation and imaging features of hemorrhagic MPE. Conclusions: We described a rare case of MPE in the lumbosacral region of a patient who presented with acute neurological compromise and atypical imaging features.