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Congenital dermoid cysts are benign developmental lesions and are a well-recognised entity in head and neck surgery. First reported in 1828 by Berger et al., these lesions have been referred to as dermoids, hairy polyps, teratoid tumours with varying classification systems. This has caused confusion in the medical literature with a lack of consensus regarding optimal diagnosis and management. Within the Eustachian tube, there have been 16 reported cases in the English literature demonstrating the rarity of these lesions. The aim of this report is to present a case of histopathologically confirmed Eustachian tube dermoid, confirm the current classification system and demonstrate the challenges they can pose given the relative inaccessibility of such lesions., (Crown Copyright © 2013. Published by Elsevier Ireland Ltd. All rights reserved.)