Large cell neuroendocrine carcinoma (LCNEC) is a rare but distinct entity of nasal cavity carcinomas. Only a very few cases have been reported in the nasal cavity. Its treatment is not well established. We report the case of a LCNEC in a 70 years old patient who presented with right nasal obstruction and epistaxis since 2 months. Diagnosis was confirmed by histological and immunohistochemical study. The patient underwent complete endoscopic removal of the tumor combined to adjuvant chemoradiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission. We will discuss in this paper the various epidemiology, clinical features, pathological findings, differential diagnosis, and evolution of treatment of this uncommon malignancy in the light of current knowledge. Optimal treatment strategies are yet to be determined for this rare malignancy with poor prognosis including surgery and chemoradiotherapy.