This article reports a very rare case of ganglioneuroma within the mandible. The patient, 15 years of age at first diagnosis, has now had 17 years of followup and remains generally healthy, despite the continued presence of the tumor. Possible origin of the lesion, histologic differentiation of this tumor from normal ganglia, and recommendations for management are discussed. Two reported cases of ganglioneuroma that occurred centrally in the mandible are also reviewed.