Abstract BACKGROUND Spinal myxopapillary ependymomas are a rare variant of spinal ependymoma that are considered to occur exclusively in the conus medullaris and filum terminale. They account for about 13% of all spinal ependymomas and have a slight male predominance. Case presentation: We report a case of 38-year-old male, Muganda, that presented at the Neurosurgery department Mulago National Referral Hospital with progressive paresthesia and paraparesis, of the upper limbs and chronic neck pain for 2years. MRI showed an isointense lesion on TIWI extending from C4 to T2 levels and exhibited heterogenous enhancement on gadolinium administration. The lesion was hyperintense on T2WI with a syrinx above the tumor. A radiological diagnosis of an intramedullary neoplastic process was made with differentials of Ependymoma, Schwanomma, and Astrocytoma. The patient underwent posterior laminectomy, and the tumor was completely excised. He is currently under follow up by the Neurosurgery team. CONCLUSION This case underscores the rarity of intramedullary myxopapillary ependymomas highlighting diagnostic challenges and the critical role of tailored surgical interventions in managing rare spinal neoplasms.