Craniofacial microsomia (CFM) is a common craniofacial anomaly characterized by asymmetric malformation of the mandible, ear, and other structures including the upper airway. The degree of mandibular hypoplasia is classified using a scheme developed by Pruzansky and modified by Kaban. Severe condylar dysplasia is the hallmark of Pruzansky–Kaban types IIB and III. Distraction osteogenesis has emerged as a treatment modality for mandibular hypoplasia in this setting for the treatment of asymmetry and airway obstruction. However, its use to resolve upper airway obstruction and avoid tracheostomy in infants with types IIB and III is not reported in the literature. The authors present a patient with successful distraction osteogenesis in a patient with CFM resulting in a Pruzansky IIB micrognathia. The authors achieved resolution of upper airway obstruction and avoidance of tracheostomy.