This is a report of a primitive neuroectodermal tumor (PNET) arising from the lingual nerve of a 5-year-old girl. Twelve months after surgical excision and postoperative chemotherapy, the patient remained alive without any evidence of recurrence or metastasis. The occurrence of PNETs in the head and neck region in children is rare. This is the first case report in the literature of a PNET originating from a cranial nerve in a child. Because of its aggressive nature and poor prognosis, aggressive surgical and medical treatment regimens are needed. Surgeons should be mindful to include PNET in the differential diagnosis of head and neck tumors.