Solitary median maxillary central incisor (SMMCI) coexists in 34%-65% of patients initially diagnosed with congenital nasal pyriform aperture stenosis. SMMCI, a genetic syndrome, warrants consideration for further screening because of its high prevalence of other diagnostic possibilities—specifically central defects, like nasal obstruction and hypothalamo-pituitary axis abnormalities. We report on a presentation of SMMCI with congenital nasal pyriform aperture stenosis which highlights the unique radiologic features and notes the relationship between these two central associated findings in the literature.
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