A rare case of hydatid cyst of the skull is presented. The presenting symptom was an acute increase of the intracranial pressure. The patient was operated upon under emergency conditions with the diagnosis of a cystic lesion of the brain. After a brief review of the literature, the symptomatology of the disease is discussed. It is emphasized that radiological studies, especially plain x-ray films of the skull, are most important in diagnosing the disease.