A case of ganglioneurocytoma of the spinal cord in a 51-year-old man is reported. Patient presented with a progressively worsening back pain and hyposthenia of lower limbs. Magnetic resonance imaging of the spinal cord revealed an intradural lesion in the T10-11 spinal segments, hyperintense on T1-weighted sequences. Complete resection was achieved. The patient did not show evidence of recurrence 12 months after surgery. On histology, the lesion was composed of round to oval cells with fine granular, slightly eosinophilic to clear cytoplasm suggesting a neurocytic differentiation. Some ganglion cells were observed. Neither significant cytologic atypia nor mitoses were present. The neoplastic cells were strongly positive with synaptophysin and negative with glial fibrillary acid protein. Neurocytomas are uncommon tumors typically arising within the lateral ventricles near the foramen of Monro. Extraventricular neurocytomas have been reported at various sites with rare examples in spinal cord. In the present paper, a case of ganglioneurocytoma of the spinal cord is reported with review of literature and discussion of differences compared to neurocytomas of other sites.