Neuroendocrine adenomas of the middle ear are rare tumors that represent less than 2% of primary tumors of the ear. In this paper, we describe a case of a 40-year-old woman who developed neuroendocrine adenoma of the middle ear. The specific management strategy for this rare tumor is unclear; information in the available literature on the management of this tumor is varied. However, an extensive demolition seems to be the gold standard treatment for this tumor to avoid recurrence and regional metastases in the lymph node or distant metastases. For the present case, we performed an incisional biopsy to confirm the diagnosis, and thereafter, we performed a canal-wall-down tympanoplasty. For cases like the present one, careful long-term clinical and instrumental follow-up is required to monitor progress and facilitate patient recovery.