Nasopharyngeal carcinoma (NPC) is a relatively rare tumor in the pediatric population, accounting for less than 1% of all pediatric malignancies. Most prevalent in teenagers than in children under 10 years of age, NPC is also highly related to the Epstein-Barr virus (EBV). NPC initially presents with nonspecific signs and symptoms such as nasal congestion, epistaxis, and/or headaches in a previously healthy child. Due to its nonspecific presenting signs, NPC is often diagnosed at a loco-regionally advanced stage in the pediatric and adolescent populations. However, it carries a better prognosis in children than in adults, with a 5-year overall survival reportedly close to 90%. NPC is sensitive to chemotherapy and radiation therapy, and concurrent chemoradiotherapy with or without neoadjuvant chemotherapy is considered as the first-line treatment in children, while surgery is only recommended for recurrences or nonresponding cases.