Hydatid disease is a parasitic infestation caused by Echinococcus granulosus, affecting both children and adults. Its intracranial form is extremely uncommon and can present a diagnostic and therapeutic challenge.

To analyze the clinical manifestations, radiological features and surgical outcomes in 21 pediatric patients with intracranial hydatidosis and to share our experience managing this pathology, with a review of relevant literature.

We report a case series of 21 pediatric patients operated on for intracranial hydatid cyst in the neurosurgical department of Ibn Tofail Hospital, Mohammed VI University Hospital, Marrakech, Morocco, between January 2012 and December 2019. We performed a comparison of our results with results from 18 case series in a review of the literature.

The most common presenting symptom was intracranial hypertension, followed by neurological deficit. Other symptoms included visual impairment, cranial nerve palsy and seizures. A computed tomography scan confirmed the diagnosis in all patients. Serological analysis was positive in 5 patients, all of whom had an associated extracerebral location. All patients were operated on successfully using the Arana Iniguez technique.

Intracranial hydatid disease should be suspected in case of intracranial hypertension in children and adolescents from endemic countries. The diagnosis is confirmed by head CT. Treatment is mainly surgical and prognosis is favorable. Prevention is the most effective way of addressing this disease and we therefore stress the importance of investing in population education and animal control.