Purpose of Review <p>Pediatric nasopharyngeal carcinoma (NPC) is a rare epithelial malignancy strongly associated with Epstein–Barr virus infection and typically presents at an advanced stage due to its deep anatomical location and nonspecific symptoms. The purpose of this review is to summarize current evidence regarding epidemiology, molecular pathogenesis, diagnostic advances, and contemporary treatment outcomes in pediatric NPC.</p> Recent Findings <p>Recent studies confirm that pediatric NPC is predominantly an undifferentiated non-keratinizing carcinoma and is almost universally associated with EBV infection. Advances in diagnostic methods, particularly MRI, PET-CT, and plasma EBV DNA quantification, have improved disease detection and staging. Multimodal treatment achieves high survival rates but is frequently associated with long-term complications, including endocrine disorders, hearing impairment, neurological deficits, and secondary malignancies. Emerging modalities such as proton therapy and immunotherapy are under investigation but require further evaluation in pediatric populations.</p> Summary <p>Pediatric NPC remains a challenging disease due to delayed diagnosis and the substantial burden of long‑term treatment toxicity. Nevertheless, survival outcomes with modern multimodal therapy are highly favorable. Continued refinement of diagnostic biomarkers, radiotherapy techniques, and systemic therapies is essential to reduce morbidity, personalize treatment intensity, and improve long‑term quality of life for affected children.</p>