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The aims of this article are to discuss the epidemiology, pathophysiology and clinical phenomenology of neurosarcoidosis, as well as current approaches to diagnosis and treatment. This review focuses on central nervous system (CNS) complications of sarcoidosis.Neurosarcoidosis is a rare disorder with diverse clinical manifestations and outcomes. It is often difficult to diagnose and even more difficult to treat. New diagnostic approaches include the use of [18F]-fluorodeoxyglucose PET to identify potential biopsy sites. Success has been reported in the treatment of steroid refractory cases with disease-modifying therapies that were originally designed to manage other chronic inflammatory conditions by neutralizing key cytokines or depleting leukocyte subsets.The diagnosis and management of neurosarcoidosis can be challenging. Currently, the disorder is treated with corticosteroids in combination with global immunosuppressant agents and/or immunomodulatory monoclonal antibodies, such as infliximab. The development of novel CNS penetrant drugs that are particularly effective at inhibiting granuloma formation would represent a significant therapeutic advance. Future progress will be informed by a deeper understanding of the pathways underlying the granulomatous inflammation characteristic of sarcoidosis and by an increased appreciation of how sarcoid lesions evolve in the CNS microenvironment.