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Intramedullary tuberculoma (IMT) is a rare form of spinal cord tuberculosis (TB). Unlike Pott’s spine, IMT is without osseous involvement and is indolent. These features may account for why the diagnosis is often overlooked as a cause of compressive myelopathy. Our case is unique in that we discuss an unusual presentation of a patient who presented with gait disturbance as the first symptom of spinal cord TB without foci of TB infection elsewhere. The patient’s neurological symptoms improved with surgery and multidrug-resistant antituberculosis treatment. Although MRI is the preferred modality to characterize IMT, findings may be nonspecific, ultimately requiring biopsy. When IMT is diagnosed and managed appropriately, it carries a good prognosis. An interdisciplinary approach would provide the best outcomes.