Gangliogliomas are glioneuronal tumours of the young, and occur more frequently supratentorially. Among those uncommon cases in the spinal cord, the conus medullaris is an extremely rare site. We reviewed the literature of such cases and report another case of a ganglioglioma of the conus in a 13-year-old boy with an insidious sensory sensitive and motor deficit of the right lower limb. Subtotal resection was accomplished with no recurrence after two years of follow-up. As for the supratentorial locations, the extent of surgical resection is the main prognostic factor for gangliogliomas of the spinal cord, and they should be considered in the differential diagnosis of intramedullar neoplasm.