OBJECTIVE To provide an up to date review of the epidemiology, etiology, treatment and prevention of hydatid cyst of the central nervous system, with an emphasis in South American countries. METHODS We searched the PubMed databases for articles containing the terms 'hydatid cyst' and 'nervous system'. RESULTS We found and reviewed 303 articles and their related references. We provide the available information on the pathology, life cycle and diagnostic modalities (laboratory tests and imaging) along with possible complications of the disease. Current treatment methods of hydatid cyst in the brain and spine are also being discussed. DISCUSSION Echinococcosis is a rare parasitic disease, endemic in some parts of South America. The epidemiology of echinococcosis is influenced by agricultural, educational, economic, medical and cultural factors. Human infestation occurs through the fecal-oral route. Infection of the central nervous system is rare, and the mainstay of treatment is surgical excision of the intracranial or spinal cyst, when present. Preventive programs should break the parasite life cycle and also educate the farmers in endemic areas.