UNLABELLED: Middle ear adenoma, a rare benign tumour with glandular and neuroendocrine differentiation, originates from the epithelial lining of the middle ear.CASE REPORT: We report a case of a 52-year-old woman, who presented with progressive hearing loss and fullness in the left ear for 3 months. Clinical examination revealed a mass in the left middle ear. Histological examination revealed tumour cells forming gland-like and cribriform structures, as well as compact groups. The nuclei were round and uniform, without atypia or mitotic activity. On immunohistochemical staining, the tumour cells were positive for epithelial (cytokeratins, epithelial membrane antigen) and neuroendocrine (neuron specific enolase, synaptophysin, chromogranin and pancreatic polypeptide) markers.CONCLUSION: Middle ear adenoma is a benign tumour that is treated by complete surgical removal. Follow-up of the patient is essential in order to detect possible recurrence. The immunohistochemical staining of the present case supports the suggestion that this tumour is best described by the term neuroendocrine adenoma of the middle ear.