Objective We retrospectively investigated spinal magnetic resonance imaging (MRI) manifestations at neurological onset in Japanese patients with spinal cord sarcoidosis. Methods Between July 2000 and April 2012, we reviewed our database and recruited patients with spinal cord sarcoidosis. On spinal MRI performed at neurological onset, the following items were evaluated: the vertebral-segment distribution and length of intramedullay T2-elongated lesions, abnormal enhancement patterns and distributions and the concomitant presence of spondylosis and associated extraspinal lesions. If available, brain MRI scans were concomitantly assessed. Results Nine patients were enrolled (four men and five women; median, 49 years). Reflecting Japanese epidemiological backgrounds, a predilection for occurrence was observed in young men and middle-aged women. Intramedullary T2-elongated lesions were present in eight patients, peaking at the C5 level, with a mean length of 3.7±2.6 vertebral segments. Spondylosis coexisted in the middle-aged patients. Abnormal intramedullary enhancement with concomitantly involved the nerve roots was observed in six patients, comprised of two types reflecting the disease progression: linear-and/or nodular enhancement along the surface of the spinal cord and intramedullary enhancement consisting of patchy, broad-based enhancement adjacent to the cord surface. Five patients had associated extraspinal lesions, including lymphadenopathy in four patients and brain involvement in four patients. Conclusion Spinal cord sarcoidosis exhibits a predilection for young men and middle-aged women among Japanese individuals and is characterized by intramedullary T2-elongated lesions spreading more than three vertebral segments peaking at the C5 level, two types of abnormal intramedullary enhancement reflecting disease progression, frequent nerve root involvement and lymphadenopathy.