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Introduction: Echinococcosis is the most common cestode infection globally caused by the Echinococcus species. The most common organ involvement is the lungs and liver, but other organs can also be rarely involved. Here, we present a case with a giant cerebral hydatid cyst. Case report: A 4-year-old boy presented with abnormal gait and walking. Cranial magnetic resonance imaging showed a cyst of 13 cm in diameter. The cyst was enucleated successfully with no rupture. Oral albendazole therapy was started. There was no eosinophilia, and the echinococcal indirect hemagglutination test was negative. Ultrasonography detected an anechoic cystic lesion in the liver. He was evaluated for deep-organ involvement, however, no cysts were detected in other organs. The histopathological examination was compatible with a hydatid cyst. Discussion: Although intracranial hydatid disease in children is rare, it should be considered among the differential diagnoses in patients with neurological symptoms, especially in highly endemic regions.