Introduction: Pierre Robin SEQUENCE (PRS), previously termed Pierre Robin syndrome, consists of three congenital abnormalities: micrognathia (small mandible), glossoptosis, and cleft palate. In Pierre Robin SEQUENCE, the events occur in SEQUENCE, with one defect leading to the next. PRS occurs with an incidence of 1 in 8500 to 25000. Affected newborns have varying degrees of airway obstruction, often in association with gastroesophageal reflux and feeding difficulties. Intervention is mostly aimed at restoring a patent airway.Case Reports: Airway management in the infant with Pierre Robin SEQUENCE can be very challenging because of difficulty with mask ventilation and intubation. Our patient was a 4-year old boy with Robin SEQUENCE scheduled for dental repair under general anesthesia. He had a history of palate repair 3 years ago. After inhalation induction with sevofluran, nitrous-oxide in oxygen‚ under spontaneous ventilation, initial laryngeal view with the conventional Macintosh laryngoscope revealed Cormack-Lehane grade IIӀ due to restricted mouth opening and micrognathia. Nasal intubation for two times under the spontaneous respiration were unsuccessful. We fiberoptically intubated the patient with uncuffed tracheal tube (ID 5 mm) while allowing him to breathe spontaneously. Extubation was done with the patient fully awake and with emergency airway equipment immediately available. Postoperatively, he was transferred to intensive care unit where he could be observed closely for delayed complications of airway obstruction.Conclusion: Managing the airway of patients with craniofacial abnormalities can potentially be difficult. Therefore we recommend even simple treatment procedures to be carried out by experienced anesthesiologists, with a variety of different airway devices available such as flexible fiberoptic‚ Storz video laryngoscope and Airtraq optical laryngoscope.