Inflammatory pseudotumor includes a diverse group of lesions characterized by inflammatory cell infiltration and variable fibrotic responses. It is extremely rare in the middle ear alone. A 7-year-old girl presented right hearing impairment. Because an otitis media with effusion was first suspected, a myringotomy was performed, but it found a mass that was different from a congenital cholesteatoma. Canal wall-down tympanomastoidectomy removed the mass successfully. The pathologic study of the specimen confirmed an inflammatory pseudotumor. We report an extremely rare case of the inflammatory pseudotumor in the middle ear with a review of the poor literature about this subject.