The purpose of this report is to review the available Literature on the pre
sentation, pathology, and treatment of central nervous system (CNS) neurocy
tomas. A case report of an extraventricular neurocytoma is presented along
with a comprehensive literature search of patients with a diagnosis of CNS
neurocytoma. CNS neurocytomas are rare neoplasms, with fewer than 240 cases
reported in the literature. The majority of neurocytomas are found in the
ventricular system of the brain. Immunohistochemistry is frequently used to
help distinguish this tumor from other CNS neoplasms. MIB-1 proliferation
index is commonly used in an attempt to predict biologic behavior. Little i
s known about the management of patients with this tumor, because most repo
rts are from the pathologic literature and contain sparse information regar
ding clinical management. Neurocytomas are rare CNS tumors with varied biol
ogic behavior. MIB-1 index may help direct adjuvant therapy. An excellent p
rognosis can be expected if a gross total resection is achieved. Postoperat
ive radiation therapy CRT) may be considered after subtotal resection. Othe
rwise, RT is an option for medically inoperable or recurrent disease.