The authors report the case of a 58-year-old man presenting with a 3-year history of clinical signs of progressive cervical spondylotic myelopathy (CSM). Magnetic resonance imaging showed a severe stenosis of the cervical spinal canal at C3-4 and C5-6 levels due to multiple discopathies. High signal intensities on T2-weighted MR images of the spinal cord and low signal intensities on T1-weighted images at the C2-6 levels were noted, as was contrast enhancement at the C3-4 level. The patient underwent a bilateral decompressive laminectomy at C3-6. The patient did not show any clinical improvement. Thus, further cervical MR imaging was performed and the differential diagnosis of an intramedullary tumor was considered in view of the persisting intramedullary enhancement. This diagnosis prompted a second operation involving a posterior midline myelotomy and excision of an intramedullary ependymoma at the C3-4 level. Intramedullary tumors should be considered in the differential diagnosis of CSM with an atypical pattern of MR imaging features.