Teratomas are germ cell tumours usually found in the young and are characteristically composed of tissue foreign to the place where they arise. Two teratomas of the middle ear were first described in 1866 and since then, and to the best of our knowledge, 19 additional cases have been reported in the literature under different terms such as hairy congenital polyps, epidermoid cysts, dermoid cysts, hamartoma, cutaneous teratoma and teratomas. The difficulty in classifying germ cell tumours may explain the different terminologies encountered in the literature. The authors describe a case of teratoma of the eustachian tube presenting as a mass in the middle and external ear of a 10-week-old girl. Using this case, a review of the literature is performed in light of the new classification of germ cell tumours proposed by the World Health Organization (WHO). It is of note that most of the teratomas that present in the middle ear arise from, or involve, the eustachian tube.