A case of intramedullary spinal cord sarcoidosis with slowly progressing symptoms and signs attributable solely to the spinal cord is reported. Biopsy disclosed granulomatous foci but was not diagnostic of sarcoidosis. However, autopsy showed numerous minute granulomas compatible with those of sarcoidosis disseminated in the lower cervical and upper thoracic cord in addition to a few in the hilar lymph nodes and papillary muscle of the heart. Clinical and pathological features of eight cases of sarcoidosis without symptoms other than those of the spinal cord are reviewed from the literature and clues to the diagnosis are discussed.