Sarcoidosis affects the nervous system in 10% of cases. When it does so it can affect any part of the nervous system and with all degrees of severity. It forms part of the differential diagnosis in inflammatory, infective, neoplastic and degenerative neurological diseases and may be very difficult to diagnose without histological confirmation. Recent clinical studies and the increasing availability of new biological treatments allow a much clearer understanding of the disease. This review summarises its clinical features, imaging and laboratory characteristics, treatment and outcome.