Neurosarcoidosis is a diagnostic consideration in diverse clinical settings. Efforts should be made to secure pathologic confirmation of systemic sarcoidosis; only rarely is central nervous system (CNS) pathologic confirmation available. CNS infection and malignancy should be reasonably excluded before making a diagnosis of CNS sarcoidosis. Corticosteroid therapy alone may not be sufficient to treat neurosarcoidosis; adjunct immunosuppressive agents are increasingly used to achieve an optimal clinical outcome.