Although neurosarcoidosis seems to occur in only 5% to 10% of patients who have sarcoidosis, it may lead to significant complications. The diagnosis of neurosarcoidosis usually relies on indirect information from imaging and spinal fluid examination. Although MR imaging remains the most sensitive technique for detecting neurologic disease, other tests, including positron emission tomography scanning and cerebral spinal fluid examination, can provide important information. The role of immunosuppressive agents such as methotrexate, cyclophosphamide, and azathioprine has been expanded, and these agents should be considered for the treatment of some manifestations of neurosarcoidosis. Reports of the antitumor necrosis factor agent infliximab suggest that this drug can be helpful for patients who have neurosarcoidosis.