Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Though first described as a cutaneous disorder, it commonly affects the lungs and other organs. Neurologic manifestations occur usually as a part of the spectrum of the systemic disease. Though relatively uncommon, neurosarcoidosis is a serious disease associated with poor outcome. Much progress has been made in the understanding of the immunopathogenesis, but there is limited knowledge about appropriate therapy, and very little is known of its etiology. The epidemiologic features, immunopathogenesis, and immunomodulatory therapy of neurosarcoidosis do not differ from the systemic disease. Most of the appreciation of neurosarcoidosis is gained from studies involving patients with systemic sarcoidosis, individual case reports, and case series of patients with nervous system disease. Patients with sarcoidosis do not generally undergo neurologic evaluation unless neurologic manifestations are prominent. Most patients with neurosarcoidosis also have other organ systems involved by the inflammatory process. In light of these reasons, it is important to understand the general features of sarcoidosis to evaluate and manage patients with neurosarcoidosis.