Spinal cord ependymomas are the most common neuroepithelial tumors in adults accounting for two-third of all spinal gliomas. Although most patients with spinal cord tumors present with typical symptoms, the presence of a nonspecific clinical presentation with low clinical suspicion results in delayed diagnosis. We report a case of spinal intramedullary ependymoma in a 39-year-old man who presented with atypical pain over the lower half of the right side of the face for 3 months. Magnetic resonance imaging revealed a high cervical intramedullary contrast-enhancing solid mass lesion. The lesion was excised via cervical laminectomy. Histopathological examination confirmed the presence of an ependymoma. Complete resolution of facial pain was achieved postsurgery. At 2 years of follow-up, there was no evidence of clinical or radiological recurrence. Atypical facial pain can be the presenting feature of spinal intramedullary ependymoma. Complete relief of facial symptoms can be achieved by microsurgical excision.