Neurosarcoidosis is an uncommon disorder and requires a careful clinical evaluation to reach a diagnosis. Generally, patients with peripheral symptoms, which include paresthesias, muscle weakness, and stocking glove deficits, have a better outcome compared with those with central nervous system involvement. Patients with mass lesions or hydrocephalus tend to have more relapses and are often more resistant to routine therapy. Neurosarcoidosis often responds to glucocorticoids, usually within days or weeks of initiating therapy. Patients are usually maintained on 40 to 80 mg per day for 4 to 6 weeks, which is then tapered slowly. Alternative treatments for refractory neurosarcoidosis, or to reduce or eliminate steroids, include methotrexate, cyclophosphamide, azathioprine, cyclosporine, infliximab, chlorambucil, chloroquine, and hydroxychloroquine.