Neurosarcoidosis is an uncommon disorder that requires careful clinical evaluation to reach the diagnosis. It may involve the CNS and peripheral nervous system and in the majority of cases it is associated with systemic disease. The demonstration of the activity of the immune system, as suggested by routine and immunopathological studies, forms a theoretical basis for the common forms of immunosuppressive therapies that are employed in sarcoidosis. Classic treatment consists of steroids either intravenously or orally. In refractory cases or in order to reduce or eliminate steroids, methotrexate, cyclophosphamide, azathioprine, cyclosporin, chlorambucil, chloroquine and hydroxychloroquine use may be an option.