A total of three cases of childhood central nervous system (CNS) echinococcosis with spinal or posterior fossa involvement having rare localizations, have been presented. Case presentation: A 7-year-old boy with spinal involvement applied to our hospital with left leg weakness and inability to walk. A 16-year-old girl with posterior fossa involvement had peripheral facial paralysis, while a 9-year-old girl had headache and vomiting. These cases, diagnosed with cystic echinococcosis with the necessary laboratory and imaging methods, improved with appropriate medical treatment and surgery. Discussion: Especially in endemic areas, it should be kept in mind that cystic echinococcosis may present with CNS involvement and should become to mind in the differential diagnosis when neurological findings are detected. Lay Summary: With this report, we attempted to share our experience with cystic echinococcosis in the spinal region and posterior fossa in three children over 1 year, including a description of associated signs and symptoms as well as laboratory and radiological findings. It should be kept in mind that cystic echinococcosis may present with CNS involvement in endemic areas and should be considered in the differential diagnosis when neurological findings are detected. Afterward, the diagnosis should be confirmed with appropriate laboratory and imaging methods. In these cases, rapid recovery can be achieved with appropriate surgical intervention as well as medical treatment.