Neuroendocrine carcinoma (NEC) of the nasopharynx is a rare and highly heterogeneous malignant tumor, whose clinical characteristics remain poorly elucidated. Unlike conventional nasopharyngeal carcinomas, NECs are not typically associated with Epstein-Barr virus (EBV) infection and often follow an aggressive clinical course. Owing to the scarcity of reported cases and limited long-term follow-up data, their prognosis remains difficult to assess. In this report, we present a case of EBV-positive small-cell neuroendocrine carcinoma of the nasopharynx. The patient was treated with six cycles of chemotherapy using the Carilizumab-EP regimen (etoposide and cisplatin), followed by concurrent chemoradiotherapy. Significant remission was achieved after this comprehensive treatment, and the patient was subsequently discharged. This case contributes to the understanding of neuroendocrine tumors in the nasopharynx and provides relevant clinical data to inform future treatment strategies.