pre-printSarcoidosis can involve the central nervous system in approximately 5-15% of cases. Any part of the nervous system can be involved, so presentation can be quite varied. Isolated disease of the spinal cord is even less common and reports are limited to single cases and small series. Although in the setting of systemic disease the diagnosis can be made with skin or lymph node biopsy, isolated disease of the spinal cord presents a diagnostic challenge. We present a case of isolated intramedullary neurosarcoidosis of the distal thoracic spinal cord presenting with posterior column dysfunction. Imaging demonstrated T2 changes in the patient's lower thoracic cord adjacent to disc herniation. Over time, however, his symptoms progressed despite decompression, and the abnormal region began to exhibit focal contrast enhancement. The persistence of symptoms as well as the new enhancement led us to perform a spinal cord biopsy, which demonstrated histopathological findings consistent with sarcoidosis. Further workup failed to reveal any evidence of systemic disease. Intramedullary sarcoidosis without systemic sarcoidosis is extremely rare. With its variable imaging appearance and inconsistent clinical manifestations, it can be difficult to diagnose. It should be considered in the differential diagnosis of a mass-like intramedullary lesion with progressive symptoms. Biopsy with histopathological correlation may be the only definite management option