Introduction: Central neurocytoma is a rare WHO grade II CNS tumor arising from the third and lateral ventricles. Extra ventricular location has been classified as a different entity in 2007 by the WHO. From it first description in 1989 around one hundred cases has been published in the literature with different protocol management.

Case report: A 21 years old man was admitted with signs of high intracranial pressure signs. The MRI showed a large right temoporoinsular tumor. The lesion displayed calcifications and surrounding edema with important brain shift. The second day of admission the patient developed signs of uncal herniation with mydriasis and decrease of consciousness so an emergent craniotomy and removal of the lesion was performed. A solid greyish lesion was removed and the pathologic examination showed findings of central neurocytoma with synaptophysin positivity and atypical changes. Gross total resection was achieved. Extraventricular central neurocytoma was diagnosed. Due to the atypical histopathological changes the patient underwent adjuvant radiotherapy and chemotherapy. Since the first surgery the patient had two recurrences, one and two years after the first surgery that were managed with surgery.

Discussion: The extra ventricular location is a rare location for central neurocytoma. This tumor could show aggressive behavior with recurrences and rapid growing. There is a lack of consensus management due to the small number of cases reported. Our treatment protocol included surgery and adjuvant radiotherapy and chemotherapy.