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简介：AbstractIntroduction:Congenital nasal pyriform aperture stenosis (CNPAS) is a rare congenital condition of structural nasal obstruction. Respiratory distress, stertor, and poor feeding are often presenting features.Case Presentation:We report a case of a newborn diagnosed with CNPAS at 3 weeks of life. The diagnosis was missed on a nasoendoscopy at day 3 of life but was realised following a facial CT when the infant presented with ongoing symptoms of upper airway obstruction. Nasal dilation was performed successfully.Conclusion:CNPAS should be considered in any neonate with upper airway obstruction. A normal nasoendoscopy does not exclude the diagnosis.