Objectives: We report a case of low-grade papillary adenocarcinoma with unique oncocytic features arising in the ethmoid sinus of a 72-year-old female. Methods: A review of the case history, pathological findings, and medical literature is performed. Results: A 72-year-old female presented with a 2-month history of progressive right-sided nasal obstruction. Preoperative nasal endoscopy showed a glistening, pink-red, soft mass arising from the middle meatus suspicious for inverting papilloma. Paranasal CT revealed a soft tissue density opacifing the right ethmoid sinus with ipsilateral maxillary and frontal sinus opacification. Complete endoscopic sphenoethmoidectomy was performed. Pathological examination of the surgical specimen revealed a low-grade papillary adenocarcinoma with oncocytic features. Special staining for MIB-1, p53, and Ki-67 was confirmatory. One year following surgical resection, nasal endoscopy revealed a recurrent mass in the surgical field; paranasal CT was confirmatory. A completion external and endoscopic ethmoidectomy was performed with complete removal of the neoplasm. Pathological examination revealed recurrent low-grade papillary adenocarcinoma. At 3 months postoperatively, the patient remains free from recurrent neoplasm. Conclusion: The management of ethmoid adenocarcinoma is dependent on the extent of disease and its pathological characteristics. Locally aggressive low-grade neoplasms, such as inverting papilloma, have been successfully treated with complete endoscopic resection with good rates of local control. Similarly, low-grade papillary adenocarcinoma has been successfully treated with endoscopic resection. In this case report, a rare oncocytic form of papillary adenocarcinoma is described that behaves similarly to other low-grade sinonasal papillary adenocarcinomas. Meticulous endoscopic surgical resection must be performed as local recurrence is common.