Sarcoidosis is a granulomatous disorder of unknown cause that affects the spinal cord in fewer than 1% of patients who suffer from it. We conducted a retrospective case-control study of 31 patients with spinal cord sarcoidosis and compared them to 30 patients with myelopathies of other causes to analyze their clinical, laboratory, and magnetic resonance imaging (MRI) profiles and to assess their long-term prognoses. Thirty-one patients presented with clinical signs of myelopathy and were diagnosed with sarcoidosis. Twenty-two of these patients had biopsy-proven noncaseating granulomas. In 9 patients, sarcoidosis involved only a neurologic localization. Patients in the control group were mainly diagnosed with multiple sclerosis or optic neuromyelitis. Patients with sarcoidosis were more likely to have elevated levels of C-reactive protein (CRP), elevated lactate dehydrogenase (LDH), and hypergammaglobulinemia in serum, as well as a higher protein content and white blood cell count in cerebrospinal fluid. Spinal cord MRIs performed in 26 patients with spinal cord sarcoidosis revealed T2-hyperintensities that were extensive and heterogeneous with a central distribution in axial slides. Twenty-six patients with spinal cord sarcoidosis presented neurologic sequelae after follow-up (mean, 64 +/- 8 mo), although 2 patients completely recovered. Neurologic sequelae correlated with cerebrospinal fluid white blood cell counts. One-third of the patients had a monophasic course of the disease, another third had a relapsing-remitting course, and the remaining third had a progressive course. Four patients had pulmonary embolism during follow-up. Spinal cord sarcoidosis remains a diagnostic dilemma since neurologic localization is frequently the only manifestation. Because treatment for spinal cord sarcoidosis is far different from treatment for other myelopathies, such as multiple sclerosis and optic neuromyelitis, diagnosis of sarcoidosis remains an important challenge. Here, we show that spinal cord MRI and blood and cerebrospinal markers may be useful tools in the diagnosis of spinal cord sarcoidosis. We suggest that accessory salivary gland biopsies, chest X-rays, protein electrophoresis, and blood levels of CRP and LDH should be obtained for each patient with subacute myelopathy. We also recommend paying careful attention to thromboembolism in patients with spinal cord sarcoidosis because of systemic disease and their decreased mobility.