This study reports a case of mucoepidermoid carcinoma (MEC) of the external auditory canal, which to date has only been described once in literature. Because the lesion is extremely rare, it is particularly difficult to classify it into stages following normal diagnostic parameters. This obviously limits the possibilities of treatment that consequently are either empirical or based on those of squamous cell carcinoma. The problems in the diagnosis and the possible methods of treatment of mucoepidermoid carcinoma are discussed.