Introduction: The neuroendocrine carcinomas may emerge in almost every body organ, though found more often on the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinus are rarely affected. The presentation age varies from 26 to 86 years old, with no sex preference. Little is known about the predisposing factors, including smoking. The treatment includes the surgical removal of the lesion, chemotherapy, and radiotherapy.