Congenital nasal pyriform aperture stenosis (CNPAS) is the narrowing of the narrowest part of the nasal cavity, which is an uncommon cause of neonatal nasal obstruction. The incidence is estimated to be approximately 1 in 25,000 live births. It arises due to an excessive growth of bone in the medial nasal process of the maxilla around the fourth month of gestation. CNPAS may present on its own or in conjunction with other anomalies. Infants, being obligatory nose breathers, commonly have nasal airway obstruction, marked by cyclic cyanosis that is alleviated when they cry. Both acute and delayed presentations are possible, exhibiting signs such as aspiration, feeding difficulties, and failure to thrive in addition to apneas, cyanosis, and respiratory distress. Symptoms may appear shortly after birth or in the first few hours of life, depending on the severity of obstruction. The diagnosis is determined through clinical assessment and imaging. Differential diagnoses include choanal stenosis and atresia, nasopharyngeal mass, and septal displacement. For mild cases of obstruction, characterized by a stable airway and no feeding difficulties, conservative treatment is generally satisfactory. Surgical intervention, commonly performed with the sublabial technique, is indicated in severe cases with an inadequate airway, unresponsiveness to conservative treatment, or poor weight gain.