We report the perioperative airway management in a 12-year-old boy suffering from Treacher Collins syndrome (TCS) and severe mental retardation who was scheduled for elective dental treatment under general anesthesia. TSC is also known as mandibulofacial dysostosis or Franceschetti syndrome, usually with a potentially difficult airway presentation. It is a major challenge for the anesthesiologist to manage an uncooperative child with such a congenital airway anomaly. A difficult airway was encountered during induction of general anesthesia, and both oral intubation by direct laryngoscopy and classic laryngeal mask airway (LMA) insertion were unsuccessful. In an expedient critical trial, with the cooperation of two anesthesiologists, one performing nasal fiberoptic intubation and the other maintaining oral mask ventilation, a nasal endotracheal tube was successfully placed at the first attempt, although at the expense of prolonged respiratory depression in the patient. Therefore, fiberoptic nasal intubation simultaneously with mask ventilation for placement of the endotracheal tube is a practical substitute for a difficult airway usually managed by LMA with inadequate ventilation. After extubation, tracheostomy may be indicated if the TCS patient suffers from persistent difficult upper airway in consequence of a traumatic intubation.