Rare diseases · Sign or symptom

Solitary median maxillary central incisor

Only one upper front tooth

HP:0006315

What it means

A single maxillary central incisor positioned in the midline with morphological symmetry of the crown and bordered by lateral incisors.

The tooth differs from a normal central incisor in the symmetric formation of the crown. The tooth is present in both deciduous and permanent dentition. Solitary/single median maxillary central incisor syndrome indicates the presence of a single median maxillary central incisor together with other midline defects of development. A single maxillary central incisor not positioned in the midline indicates agenesis of the contralateral central incisor and can be differentiated furthermore by the morphology of the crown. A diagnosis of a solitary median maxillary central incisor typically requires X-ray examinations.

Rare diseases that can present with this12

The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.

Part of the broader category

Also called: Single central incisor · Single central upper incisor · Single maxillary central incisor · Single median incisor · Single median maxillary incisor · Single midline maxillary incisor · Single midline upper front tooth · Solitary median maxillary central incisor syndrome

Solitary median maxillary central incisor

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This page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.