OBJECTIVE: This study aimed to evaluate the changes in upper airway (UA) dimensions in growing patients with Pierre-Robin sequence (PRS). METHODS: The subjects were 23 PRS patients who had not undergone growth modification therapy or surgical intervention. Their lateral cephalograms were obtained longitudinally at mean ages of 8.81 (T0) and 14.05 (T1). Patients were categorized based on their SNB value at T0 (Criteria: -2 SD): Group-1 (very retrusive mandible, n = 13) and Group-2 (moderately retrusive mandible, n = 10). Skeletal and UA variables at T0 and T1, as well as ΔT0-T1, were statistically analyzed. RESULTS: At T0, Group-1 exhibited more retrusive maxilla and mandible (SNA, CONCLUSIONS: Even in growing PRS patients with severe mandibular retrusion, the UA dimensions increased due to forward growth of the mandible, repositioning of tongue and hyoid bone, and existence of compensatory mechanism.