Neuroendocrine adenoma of the middle ear (NAME) is a rare tumor. We report a case of NAME, the clinical and pathologic findings of which illustrate the biologic behavior of adenomatous tumors of the middle ear and their relationship with rare carcinoid tumors of the middle ear. A 29-year-old man presented with a history of recurrent otitis media, right conductive hearing loss, and aural fullness. The tumor was removed in its entirety. Otolaryngologists should be familiar with this unusual but important entity.