A 30-year-old female presented with a central neurocytoma manifesting as positional headache. Magnetic resonance imaging showed a large calcified mass in the right lateral ventricle. The tumor was completely removed surgically. Light microscopy showed a linear arrangement of well-defined cells (beaded-string pattern) containing small round nuclei with abundant perikarya. A perinuclear halo reminiscent of oligodendroglioma was prominent in some cell groups. The tumor was entirely amitotic and free of atypia. Large, plump spheroid bodies lacking nuclei but containing coarse brown pigments were present among the tumor cells. The tumor cells were immunoreactive to neuron-specific enolase and synaptophysin but not glial fibrillary acidic protein or neurofilament. The ultrastructure of the tumor cells included abundant spheroid bodies containing secretory granules around neurons, indicating a dystrophic or aborted process of neuronal differentiation rather than a completely-differentiated central neurocytoma.