<p><strong>Objective</strong>. Nasopharyngeal carcinoma is an extremely rare pediatric malignancy predominantly occurring in adolescent males. Its multifactorial pathogenesis is most strongly associated with the exposure to<br />Epstein-Barr virus in genetically susceptible hosts. In younger patients, more aggressive biological behavior has been observed, although the overall survival is better compared to adults. Due to its rarity and nonspecific clinical presentation, the diagnosis in children is often delayed and misinterpreted. <strong>Case report</strong>. We report a case of a 16-year-old boy with stage IVB nasopharyngeal carcinoma. He presented with a painless palpable neck mass, nasal congestion and a history of occasional epistaxis and headaches. Four years after the completion of a multimodal treatment, the patient is in complete remission. <strong>Conclusion</strong>. Although exceedingly rare, pediatricians should consider nasopharyngeal carcinoma in the differential diagnosis of palpable neck masses, especially in male adolescents. A multidisciplinary approach in the diagnosis, treatment, supportive care and follow-up is of utmost importance.</p>

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