Central primitive neuroectodermal tumour (cPNET), a rare malignant neoplasm of embryonal origin, often occurs in children younger than 15 years. This is the first case report of the imaging findings of an infratentorial cPNET to be reported in a patient. Here, is reported the case of a 6-year-old boy presenting with symptoms of diplopia for 14 days. Magnetic resonance imaging revealed a solid mass in the fourth ventricle. The postoperative pathological diagnosis was cPNET. To conclude, whenever a child is diagnosed to have an infratentorial solid tumour in the fourth ventricle, cPNET should always be considered despite its rarity.
