The triad of glossoptosis, micrognathia, and cleft palate characterizes the Pierre Robin sequence (PRS). Complications can be severe and the clinical manifestations are very heterogeneous, but airway obstruction is almost present and may be life threatening. To avoid tracheostomy or allow for early decannulation in severely affected children, external or bioresorbable internal distraction devices can be applied to repair mandibular deficiencies. We report a case of a children submitted to bilateral mandibular distraction with external nonresorbable devices. After optimal lengthening of mandibles, laryngotracheoplasty was performed to close the tracheostomy with improvement of respiratory pattern and quality of life this child. Bioresorbable internal distraction devices offers some advantages, but complications include device failure to support resistance. Distraction osteogenesis has become a safe, less-invasive procedure with a negligible risk of infection making it effective to substitute tracheostomy as first choice in treatment of patients with Pierre Robin Sequence and life threatening airway obstruction.