Sarcoidosis is a multisystem disorder of unknown cause. The disease is characterized by the presence of noncaseating granulomas in the affected tissue system. Clinically recognizable nervous system involvement occurs in about 10% of patients with sarcoidosis, although the incidence of subclinical neurosarcoidosis is much higher. The disease appears in the differential diagnosis of many infectious and noninfectious neurological syndromes. We have reviewed all aspects of neurosarcoidosis from the point of view of practicing internists, including the clinical manifestations, cerebrospinal fluid and radiographic changes, differential diagnosis, and treatment.