ObjectivesPapillary seromucinous adenocarcinoma of the sinonasal tract is exceedingly rare. The objectives of this case report are to describe a case of papillary seromucinous adenocarcinoma presenting in the nasopharynx and to review the literature pertaining to other similar cases.MethodsA review of the patient's chart and a review of the English literature were conducted.ResultsWe describe the case of a 64 year-old woman who presented with a 3-year history of epistaxis and right-sided otitis media with effusion. The patient had been followed for a known nasopharyngeal mass that had twice been biopsied and in both cases was considered a benign mass pathologically. A third biopsy was diagnosed as a low-grade papillary seromucinous adenocarcinoma. The patient was otherwise asymptomatic. The patient was referred to a multidisciplinary cancer clinic at which endoscopic resection was determined to be the preferred treatment modality. A literature review and approach to patients with nasopharyngeal masses will be presented.ConclusionsPapillary seromucinous adenocarcinoma is a rare tumor that can present in the nasopharynx. We describe the endoscopic surgical management of one such patient that presented to our care.