Spinal cord involvement in sarcoidosis is rare. Correct diagnosis and proper treatment are important to achieve good outcome. We present a case of cervical spinal sarcoidosis with progressive myelopathy. MRI revealed a C7-T3 ill-defined intramedullary lesion with heterogeneous enhancement. Due to the patient's history of sarcoidosis involving mediastinal lymph nodes and lung, which was biopsy-confirmed, spinal sarcoidosis was suspected. Clinical and radiological improvement was obvious after steroid treatment. When confronted with an intraspinal lesion, the presence of leptomeningeal involvement should alert the clinician to the probable diagnosis of spinal sarcoidosis. Corticosteroids are the primary treatment, and the response should be dictated by clinical assessment, MRI, and neurophysiological monitoring. Biopsy or surgical resection might result in catastrophic sequelae and is rarely indicated.