Hydatid disease is a parasitic infection whose etiologic agent is Echinococcus granulosus. Human is an accidental intermediate host and the most common site is the liver. The brain involvement is unusual and up to 75% of cases are described in the pediatric population. We present six children with cerebral hydatid disease admitted to the Pediatric Hospital J.P. Garrahan. All had neurological involvement on admission. The images showed single cystic lesion in the brain. They did not present involvement in other organs. Serology was negative in all cases. Medical and surgical treatment in all cases. The clinical outcome was favorable without sequelae in five of them and one had a residual paresis right faciobrachiocrural. This infection should be considered in the differential diagnosis of cystic tumor lesions of the central nervous system.