Congenital nasal pyriform aperture stenosis (CNPAS) is a rare form of nasal obstruction in infants. It can cause respiratory distress and feeding difficulties, as neonates are obligate nasal breathers. The incidence of CPNAS seems to be underestimated. We report a case of CNPAS diagnosed in a three-weeks old neonate. She was initially started on conservative treatment based on topical nasal medication and humification, but without good clinical result. At the hospital heated humidified high-flow nasal cannula (HHHFNC) was started as a temporary treatment. After surgical treatment through sublabial approach, she could be weaned from high flow therapy.