Ganglioneuromas are rare benign tumors originating from the ganglion cells of the sympathetic and parasympathetic nervous system. Ganglioneuromas in the base of skull are extremely rare. In this article, we describe a case of primary ganglioneuroma below the foramen ovale observed in a 38-year-old man. The lesion was asymptomatic. The patient underwent surgical intervention for diagnostic and therapeutic purposes. Craniomaxillofacial surgery was conducted by the oral and maxillofacial surgical team and the neurosurgical team. Combined frontotemporal-preauricular infratemporal approach was used to expose the lesion. Cerebrospinal fluid leakage and facial paralysis did not occur postoperatively. The results of histopathologic examination indicated that the excised lesion was a ganglioneuroma. Clinical follow-up was done, and no recurrence has been observed up to now.