A 39-year-old woman had development of new-onset numbness in her left arm. This progressed over 2 to 3 weeks to involve the left axilla, trunk, lower extremities, and genital region. She had mild imbalance and left-sided weakness but remained ambulating without a gait aid. Repeated magnetic resonance imaging of the cervical spine showed the longitudinally extensive T2 lesion in a central location on axial sequences, with linear, dorsal, subpial gadolinium enhancement extending more than 2 vertebral segments. The magnetic resonance image findings were most suggestive of spinal cord neurosarcoidosis. Computed tomography of the chest showed bilateral hilar adenopathy. Serum levels of angiotensin-converting enzyme were normal. Transbronchial lung biopsy showed noncaseating granulomas. Noncaseating granulomas were confirmatory of pulmonary sarcoidosis, which led to a diagnosis of spinal cord neurosarcoidosis. Treatment with intravenous methylprednisolone for 5 days was repeated, followed by oral prednisone for 2 months. Her neurologic symptoms improved, and repeated magnetic resonance image showed a marked decrease in T2 hyperintensity and gadolinium enhancement consistent with interval response to treatment. A slow prednisone taper over 9 months was initiated. Spinal cord neurosarcoidosis often presents with an isolated myelopathy without symptoms of pulmonary sarcoidosis (eg, cough, dyspnea). The presentation can range from a subacute onset mimicking transverse myelitis (as in this case patient) to a more insidious progressive myelopathy over months to years.