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Ectodermal dysplasias are a rare group of heritable disorders involving the ectodermal derivatives with only a few published reports involving its anaesthetic management. We present the case of a 16-year-old boy with ectodermal dysplasia presented for elective oral surgery under general anaesthesia with a surgeon preference for nasotracheal intubation to provide adequate surgical exposure. The patient had successful nasal flexible bronchoscopic intubation despite challenging tracheal intubation conditions. We summarise previous case report