Background The single median incisor is a rare dental abnormality that could be isolated or could be part of many different syndromes or syndromic association with poor prognosis. Case description We report the first prenatal ultrasound description of a 31-year-old patient, gravida 1, para 0, whose male fetus was diagnosed at 25 weeks’ gestation with a single median incisor suggestive of nasal pyriform aperture stenosis in Montpellier University Hospital (France). A fetal magnetic resonance imaging (MRI) performed at 30 weeks’ gestation retrieved no intracranial midline cerebral anomalies and confirm nasal pyriform aperture stenosis suspicion. Amniocentesis, performed at 31 weeks, found a normal fetal karyotype (46XY) and a normal comparative genomic hybridization (CGH) array. After term vaginal delivery, clinical and radiological examination confirmed the diagnosis of an isolated single median maxillary central incisor linked to nasal pyriform aperture stenosis. Conclusion Prenatal diagnosis of a single median incisor due to nasal pyriform aperture stenosis is feasible and enables close postnatal follow-up. How to cite this article Fuchs F, Chadelle M, Captier G, et al. Solitary Median Maxillary Central Incisor due to Nasal Pyriform Aperture Stenosis in Fetus: The First Prenatal Ultrasound Case Report. Int J Clin Pediatr Dent 2020;13(3):295–298.