Apert syndrome is an autosomal dominant disease presenting with craniosynostosis, midface hypoplasia and syndactyly. Children with Apert syndrome usually present to the operating room for craniofacial and extremity operations. During anaesthesia, difficult intubation and ventilation is expected because of abnormal airways. In case of Apert syndrome, anaesthetists must be prepared for intubation difficulties, airway, ventilation problems and even visceral anomalies. We report a case of Apert syndrome referred to us for endoscopy of upper respiratory tract and syndactyly release. This article will focus on the difficulties and complications related to the anaesthetic management.