Neuroendocrine adenomas are rare tumors, which can appear in the middle ear. Approximately a hundred cases have been reported in the literature. We report the case of a 58-year-old man who consulted for an abnormal sensation of fullness in the right ear. The otoscopic examination showed a retrotympanic tumefaction. The CT scan and MRI of the middle ear demonstrated a well-defined tissue mass without any osteolysis. We performed surgical exeresis by transcanal procedure with a cartilage graft tympanoplasty. Microscopic examination and immunohistochemistry revealed an endocrine adenoma of the middle ear. Neuroendocrine adenomas can develop in a number of different sites. When they appear in the middle ear they usually produce hypoacousia. The otoscopic examination shows non-specific findings with only retrotympanic swelling. Surgical exeresis enables histologic and immunohistochemically analysis of the surgical specimen. The adenoma is composed of two cellular types: neuroendocrine (which closely resemble carcinoid tumors) and glandular. Regular clinical and radiologic follow-up is necessary since recurrence is possible. The formal diagnosis of neuroendocrine adenomas of the middle ear requires histologic and immunohistochemically confirmations since the clinical symptoms are non-specific. Surgical excision with removal of the ossicular chain is the treatment of choice in order to prevent recurrence.