Aim: Our aim is to highlight the successful treatment of congenital nasal pyriform aperture stenosis by expectant management and the importance to understand other disorders that may occur alongside it. Discussion: Congenital nasal pyriform aperture stenosis may occur in isolation, or as a part of the holoprosencephaly spectrum. This case report demonstrates the role of diagnostic imaging to confirm its diagnosis and to look for other concurrently occurring pathologies. Case history: We report a newborn developing respiratory embarrassment shortly after birth, requiring intubation to sustain breathing. A diagnosis of congenital nasal pyriform aperture stenosis was clinched as evidenced by the radioimaging features. The patient also had a solitary median maxillary central incisor. Conclusion: Expectant management has proved to be beneficial as exemplified in this case report. Although this rare disease may present as an isolated entity, it is paramount to be mindful of other anomalies that may coexist.