Backgroud <p>Small cell neuroendocrine carcinoma is a rare occurrence in the nasopharynx, characterized by aggressiveness and poor prognosis, with clinical and pathological features that remain incompletely understood.</p> Case presentation <p>We present a case of Epstein-Barr virus-positive nasopharyngeal small cell neuroendocrine carcinoma of a 58-year-old Chinese man who initially presented with symptoms of left ear tightness and aural fullness. The patient underwent three cycles of induction therapy comprising chemotherapy (cisplatin-etoposide) in conjunction with immune checkpoint inhibitors (adebrelimab), followed by concurrent chemoradiotherapy (triweekly cisplatin and a total dose of 68&#xa0;Gy delivered in 31 fractions). Subsequent radiological evaluation revealed a complete response post-treatment at 6&#xa0;months, with no evidence of recurrence or metastasis at the 6-month follow-up.</p> Conclusion <p>Managing nasopharyngeal small cell neuroendocrine carcinoma poses challenges due to its rarity. Treatment modalities encompass surgery, radiotherapy, concurrent chemoradiotherapy, and chemotherapy alone. Novel therapeutic strategies, such as immune checkpoint inhibitors, may offer promising options for advancing treatment modalities. Further clinical data on similar cases are warranted to enhance treatment efficacy.</p>