Rare diseases · Sign or symptom
Tooth agenesis
Decreased tooth count
HP:0009804
What it means
The absence of one or more teeth from the normal series by a failure to develop
Teeth agenesis needs to be confirmed by X-rays. Teeth agenesis encompasses hypodontia, oligodontia, and anodontia. The total number and the type of teeth missing should be added to the description. The clinical absence of a tooth due to a disturbed eruption should not be termed teeth agenesis but a missing tooth.
Rare diseases that can present with this46
Very common80–99%
21- Autosomal dominant primary microcephaly
- Brachydactyly-mesomelia-intellectual disability-heart defects syndrome
- Charlie M syndrome
- Cleft lip/palate-deafness-sacral lipoma syndrome
- Cohen syndrome
- Cutis gyrata-acanthosis nigricans-craniosynostosis syndrome
- Dental ankylosis
- Dermoodontodysplasia
- EEC syndrome
- Epidermolysis bullosa simplex with anodontia/hypodontia
- Extrasystoles-short stature-hyperpigmentation-microcephaly syndrome
- Hennekam syndrome
- Laron syndrome
- Microcephalic osteodysplastic primordial dwarfism type II
- Oculodentodigital dysplasia
- Odonto-onycho dysplasia-alopecia syndrome
- Osteoglosphonic dysplasia
- Papillon-Lefèvre syndrome
- Progeroid syndrome, Petty type
- Short stature-wormian bones-dextrocardia syndrome
- Trichodermodysplasia-dental alterations syndrome
Common30–79%
10- Amelo-onycho-hypohidrotic syndrome
- Ankyloblepharon filiforme adnatum-imperforate anus syndrome
- Branchio-oculo-facial syndrome
- Congenital unilateral hypoplasia of depressor anguli oris
- Craniofacial dysostosis-diaphyseal hyperplasia syndrome
- Focal dermal hypoplasia
- Holoprosencephaly
- Maxillonasal dysplasia
- Seckel syndrome
- Treacher-Collins syndrome
Sometimes5–29%
15- Acrofacial dysostosis, Catania type
- Alström syndrome
- Blepharonasofacial malformation syndrome
- Enamel-renal syndrome
- Kallmann syndrome
- Moebius syndrome
- Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to 9q21.3 microdeletion
- Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation
and 7 more in this range
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: Decreased number of teeth · Dental agenesis · Failure of development of some teeth · Fewer teeth than normal · Missing some teeth · Reduced number of teeth · Teeth, agenesis
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Start free with EleplanThis 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.