Carcinoid tumor of the middle ear is an extremely rare lesion of the middle ear, and thus its diagnosis is frequently delayed. Some authors found it hard to differentiate middle ear carcinoid front the middle car adenoma. However, the balance of opinion is currently oil the side of considering it as a separate clinical entity. Definitive diagnosis is made by identifying neurosecretory tumor cells using immunohistochemistry and electron microscopy. It usually follows a nonaggressive clinical course, rarely metastases, and infrequently recurs after radical excisions. We present a patient with middle ear carcinoid, who is free of disease 2 years after the resection of the tumor mass with canal walldown procedure. The relevant literature is also herewith reviewed. (C) 2008 Elsevier Inc. All rights reserved.