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Congenital nasal pyriform aperture stenosis has recently been described in the literature. It is caused by an upward overgrowth of the maxillary bone apophysis and may cause breathing and feeding problems from the neonatal period on. We present the case of a newborn girl diagnosed with this pathology associated with a solitary maxillary central incisor. Observation with conservative measures was the attitude chosen. One year after diagnosis the patient shows adequate height and weight development.