Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors that can appear in many different locations as neuroendocrine cells are distributed throughout the anatomy during embryonic development. This paper presents a case report of a 77-year-old woman with a rare NEN in the lateral wall of the pharynx. In addition to being very infrequent, it can be considered a second metachronous tumor since it is unrelated to a previous sinonasal NEN that the patient presented with 20 years before. We have reviewed the histological characteristics of NENs and the grading system used to determine their potential for metastasis or local invasion. NENs in the oropharynx are very infrequent and typically do not present systemic symptoms or specific local signs. The article concludes that surgical excision is typically the preferred treatment for localized NENs where complete removal is possible.