Cystic echinococcosis is a zoonotic infection that occurs worldwide. Humans are infected through ingestion of parasite eggs in contaminated food, water or through direct contact with infected dogs, which are the definite host. Humans serve accidentally as intermediate host, and occurrences are common in children and young adults. Cystic echinococcosis is endemic in Mediterranean, South American, Middle Eastern, Central Asia, East Africa countries and Australia. The liver is the most frequently involved organ, followed by lungs. Hydatid cysts have been reported only in 2% of cases in the brain. Primary cerebral hydatid disease is a rare entity, but should be considered in the differential diagnosis of cerebral lesions.