The adenomatous tumor, derived from the mucosal epithelium of the middle ear, is a rare neoplasm, which has been called adenoma or adenocarcinoma. Recently, the adenomatous tumor classified into mixed and papillary type. Among two always confined to the middle ear and mastoid. Immunohistochemically the tumor cells denonstrate diffuse positive reactions for neuron specific enolase and focally for chromogranin. Ultrastructual study shows numerous dense secretory granules within the cytoplasm. In this case, authors emphasize the clinical presentations, as well as radiographic and pathologic characteristics of the tumor. We present a case of the adenomatous tumor of the middle ear with a review of the literatures.