This review focuses on neurosarcoidosis with pathological alterations in the brain. Patients with neurosarcoidosis develop several symptoms such as cranial nerve palsies, headache, consciousness disturbance, and seizures. It may be difficult to reach a definite diagnosis and carry out differential diagnosis. Sarcoidosis is characterized by noncaseating granuloma that may be seen at the dura, leptomeninges, brain (including the cranial nerves), spinal cord, and peripheral nerves. Epithelioid histocytes and multinucleated giant cells, including Langhans cells, are characteristic microscopic features. In some instances, an asteroid body and a Schaumann body may be seen. In particular, infectious diseases including tuberculosis and mycosis must be ruled out. Therefore, the pathologic diagnosis of brain lesions is the gold standard for diagnosing neurosarcoidosis. In addition to prednisolone therapy, infliximab, a monoclonal antibody against tumor necrosis factor-α, has received much attention for treating neurosarcoidosis.