Neurologic manifestations occur in more than 5% of sarcoidosis patients and may be the presenting feature. Neurosarcoidosis can manifest in a myriad of ways including: cranial neuropathy, aseptic meningitis, mass lesions, encephalopathy, vasculopathy, seizures, hypothalamic-pituitary disorders, hydrocephalus, myelopathy, peripheral neuropathy, and myopathy. Because its etiology is unknown, its neurological manifestations are so diverse, and its diagnosis cannot be readily confirmed by laboratory tests, neurosarcoidosis poses many clinical problems. The diagnosis of neurosarcoidosis is usually based on the identification of characteristic neurologic findings in an individual with proven systemic sarcoidosis as established by clinical, imaging, or histologic findings. Although corticosteroids are regarded as the foundation of treatment, they are not always successful and have serious side-effects. Moreover, some patients with neurosarcoidosis are refractory to conventional therapy, and approximately 5-10% die. Optimal management of patients with neurosarcoidosis benefits from an understanding of the broad clinical spectrum of neurosarcoidosis, appreciation of the ways to best confirm a diagnosis, and awareness of the full range of treatment options, including the use of alternative therapies such as immunotherapy.