Ganglioneuromas are rare, benign neoplasms derived from sympathetic neural crest progenitor cells. In the pediatric population, ganglioneuromas usually develop in the mediastinum or retroperitoneum. We report the case of a 3-year-old boy who presented with a painless enlarging neck mass, which was found to be a parapharyngeal space ganglioneuroma that extended to the skull base. We summarize the current principles regarding the diagnostic workup and treatment of these neoplasms, and we briefly review the literature.