Middle ear salivary gland choristoma is a rare entity, and less than 30 cases have been reported since the first description in 1961 by Taylor and Martin.1 The predominant clinical features of this disease are unilateral conductive hearing loss and abnormalities of the middle ear ossicles and the facial nerve. In this case report, we present an atypical case of salivary gland choristoma of the middle ear and review the literature.