Encephalocele is a parasitic disease that occurs in the brain of the central nervous system. It is a cystic space-occupying lesion caused by the larvae of the worm entering the skull through the bloodstream and colonizing the skull. It causes increased intracranial pressure and symptoms of epilepsy. Surgery is currently the only curative approach, and the aim and principle of surgery is to remove the cyst completely, avoiding extravasation of cystic fluid that can lead to recurrence and intracranial dissemination and metastasis. Anti-parasitic drugs can be taken before and after surgery to minimize recurrence and improve efficacy. Generally the prognosis of surgery is good if the lesion can be completely removed.