Neuroendocrine carcinoma is a type of neuroendocrine system neoplasm that occurs in organisms in which amine precursor uptake and decarboxylation (APUD) cells are uncommon. In the human body, the most common sites of neuroendocrine carcinoma are the gastrointestinal tract and the lungs. From a histologic standpoint, these tumors are identified by their positive reaction to silver stains and specific markers for neuroendocrine tissues, such as chromogranin A, synaptophysin, and neuron-specific enolase. In addition to local symptoms, these tumors may cause flushing, wheezing, and episodic diarrhea secondary to the release of vasoactive substances. Our medline search encompassing the 20-year period prior to 2003 found few cases of this type of tumor in the paranasal sinuses and nasal cavities and only 1 case of an isolated neuroendocrine carcinoma in the nasopharynx. We describe another case of an isolated nasopharyngeal neuroendocrine carcinoma, which we observed in a 74-year-old woman. We review the clinical presentation and the surgical and systemic treatment.