Unlabelled: We describe an infant with popliteal pterygia, syngnathia, cleft lip and palate, and retrognathia diagnosed with popliteal pterygium syndrome (PPS). The neonatal course was complicated by severe obstructive apnea necessitating tracheostomy.Conclusion: This report illustrates the potential for airway compromise in PPS patients and the need for thorough neonatal airway assessment.