A 40-year-old man presented with right-sided nasal blockage and epistaxis of 18 months' duration. Examination revealed a pinkish mass in the right nasal cavity and nasopharynx. Initial biopsy was suggestive of angiofibroma. Complete excision through a lateral rhinotomy approach was performed. The postoperative histopathologic report was suggestive of sinonasal neuroendocrine carcinoma, which was confirmed by immunohistochemistry. To the best of our knowledge, this is the first reported case of sinonasal neuroendocrine carcinoma arising from the lateral wall of the nasopharynx in the English literature. We describe its clinical presentation and management and provide a brief review of the literature.