Study design: Case report. Objective: To report on an uncommon patient of cervical spinal cord sarcoidosis. Setting: New York, USA. Methods: A 47-year-old man was evaluated for heaviness of his legs, difficulty walking and numbness of arms and feet for 6 months. Contrast enhanced MRI scan showed a strongly enhancing nodular lesion, suggestive of a spinal cord tumor, which was initially considered for neurosurgical biopsy and treatment. Investigations unexpectedly revealed mediastinal lymphadenopathy and elevated angiotensin converting enzyme levels and the diagnosis of sarcoidosis was confirmed by a transbronchial lung biopsy. Result: Prompt treatment with steroids resulted in complete clinical and radiological resolution of the spinal cord lesion within 4 weeks. This improvement was maintained over a 18 month follow-up period. Conclusion: Isolated spinal cord intramedullary enhancing lesions, even when the appearance is suggestive of a tumor, should be rigorously investigated for sarcoidosis and a trial of steroid therapy considered to avoid unnecessary surgery.