Abstract: Nasopharyngeal carcinoma is a rare condition, accounting for < 5% of all malignant tumors in children, and genetic susceptibility may be its pathogenesis. Chemoradiotherapy is the main treatment modality for locoregional advanced nasopharyngeal carcinoma. Intensity-modulated radiotherapy with platinum-based induction chemotherapy, synchronous chemotherapy, or adjuvant chemotherapy is the preferred treatment for childhood nasopharyngeal carcinoma. After radiotherapy, 84% of patients with childhood nasopharyngeal carcinoma have different degrees of radiation injury. For now, there is still a lack of the best combination regimen of radiation dose, chemotherapy, and chemoradiotherapy to reduce complications and improve the long-term quality of life. In addition, future studies should focus on the new type of targeted immunotherapy for childhood nasopharyngeal carcinoma; moreover, further prospective multicenter trials should be conducted. This review illustrates the clinical research achievements and future research directions for childhood nasopharyngeal carcinoma.