The spinal cord is a very rare location for extra-ventricular neurocytomas. Composed of small round cells with neuronal differentiation, neurocytoma has a particularly favourable prognosis. Long disease-free intervals have been observed in patients who received sub-total removal and radiotherapy. A careful intra-operative frozen section examination is helpful in avoiding excessive surgical tissue disturbance and to identify the best treatment option. To date, eight cases of neurocytoma of the spinal cord have been described. We report three more patients; one with very long survival, and review the published literature for this condition.