Introduction: Congenital nasal pyriform aperture stenosis and solitary median maxillary central incisor are uncommon anomalies and are associated with further malformations. Solitary median maxillary central incisor itself initially has no impact on a child's health, but congenital nasal pyriform aperture stenosis is a potentially life-threatening condition. Case presentation: A Caucasian baby boy showed severe dyspnea and was intubated orotracheally. Multiple anomalies were detected, including urogenital and craniofacial malformations. Computed tomography scans revealed congenital nasal pyriform aperture stenosis with a diameter of 4.9mm and a solitary median maxillary central incisor. A 3.0mm tube was inserted in his left nasal cavity, and the baby was able to breathe sufficiently and spontaneously. The nasal tube was removed after seven days, and the baby was discharged under application of decongestant drops. After seven months, the baby was readmitted with respiratory distress, and surgery was carried out using an intraoral sublabial approach. The stenotic area of the pyriform aperture was widened, and 3.0mm tubes were inserted in both nasal cavities for 10 days. Over a period of six month, no further respiratory distress has occurred. Conclusion: The decision to perform surgery was delayed since the baby's nasal breathing was adequate as a result of the insertion of a nasal tube. Since treatment depends on the severity of symptoms, it is appropriate in some cases to take a conservative approach at first and to keep surgery as a last resort. Once a conservative approach has been selected for congenital nasal pyriform aperture stenosis, awareness of the life-threatening nature of the condition should be kept in mind, and a surgical approach must still be taken into account. Keywords: Congenital nasal pyriform aperture stenosis, solitary median maxillary central incisor, malformations, associated anomalies