Central neurocytoma is a rare neuroectodermal tumor found in young adults. These tumors are generally located in the lateral or third ventricles. Extraventricular neurocytoma in the spinal cord is extremely rare. We report on two patients with primary spinal neurocytomas who presented with progressive numbness and weakness in the limbs. Both patients had intramedullary masses between the medulla and the upper thoracic levels. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all 20 reported cases were reviewed. In conclusion, neurocytoma should be included in the differential diagnosis of a spinal intramedullary tumor, and subtotal resection is acceptable for a relatively favorable prognosis if gross total removal is unachievable. The efficacy of adjuvant radiochemotherapy to control tumor recurrence is unknown.