Background: Spinal cord contrast enhancement is an unusual radiological finding in chronic degenerative disorders of the spine and often it is misdiagnosed with neoplastic or inflammatory disease. Case Description: Here we present the case of a 62-year-old male with cervical degenerative spondylosis at the C4–C5 and C6-C7 level. Preoperative magnetic resonance imaging showed severe spinal cord compression with intramedullary hyperintensity on T2-weighted sequences and spinal cord enhancement at the C4-5 level after administration of Gadolinium. The patient with several weeks’ history of worsening spastic tetraparesis, underwent two-level anterior discectomy with fusion and anterior stabilization at the myelopathy level. Conclusion: Intramedullary gadolinium enhancement due to cervical spondylotic myelopathy (CSM) is an important consideration in the differential of inflammatory disease or intramedullary tumors, especially since misdiagnosis may result in serious consequences, including neurological disability from delayed surgical interventions and unnecessary treatments. Persistent enhancement for months to years following decompressive surgery is usual. Recognition is important to prevent inappropriate interventions or delay in consideration of a potentially beneficial decompressive surgery.