Rare diseases · Sign or symptom
Hypodontia
Failure of development of between one and six teeth
HP:0000668
What it means
The absence of five or less teeth from the normal series by a failure to develop.
Hypodontia needs to be confirmed by X-rays. The terms hypodontia and oligodontia have been used interchangeably in literature but these define two different clinical entities. Hypodontia has been used to define exclusively the absence of permanent teeth and excluding third molars, but the absence of any deciduous and permanent teeth, including third molars, should be called hypodontia as well.
Rare diseases that can present with this72
Very common80–99%
21- 4H leukodystrophy
- Acrofacial dysostosis, Weyers type
- Ankyloblepharon-ectodermal defects-cleft lip/palate syndrome
- Autosomal dominant hypohidrotic ectodermal dysplasia
- Autosomal spastic paraplegia type 58
- Böök syndrome
- Cerebellar ataxia-ectodermal dysplasia syndrome
- CHIME syndrome
- Coffin-Lowry syndrome
- Ectodermal dysplasia, trichoodontoonychial type
- Faciocardiorenal syndrome
- Hypodontia-dysplasia of nails syndrome
- Hypohidrotic ectodermal dysplasia
- Hypomyelination-hypogonadotropic hypogonadism-hypodontia syndrome
- Leukomelanoderma-infantilism-intellectual disability-hypodontia-hypotrichosis syndrome
- Monosomy 18p syndrome
- Non-eruption of teeth-maxillary hypoplasia-genu valgum syndrome
- Peroxisomal acyl-CoA oxidase deficiency
- Progeria-short stature-pigmented nevi syndrome
- Severe microbrachycephaly-intellectual disability-athetoid cerebral palsy syndrome
- Wolf-Hirschhorn syndrome
Common30–79%
21- 19p13.12microdeletion syndrome
- ANE syndrome
- Angel-shaped phalango-epiphyseal dysplasia
- Bardet-Biedl syndrome
- Cranioectodermal dysplasia
- Dyskeratosis congenita
- Ellis-Van Creveld syndrome
- Focal dermal hypoplasia
- Hypoglossia-hypodactyly syndrome
- Incontinentia pigmenti
- Kabuki syndrome
- Lelis syndrome
- Mutilating palmoplantar keratoderma with periorificial keratotic plaques
- Orofaciodigital syndrome type 1
- Postaxial polydactyly-dental and vertebral anomalies syndrome
- Schöpf-Schulz-Passarge syndrome
- Temtamy preaxial brachydactyly syndrome
- Tremor-ataxia-central hypomyelination syndrome
- Ventricular extrasystoles with syncopal episodes-perodactyly-Robin sequence syndrome
- Wiedemann-Rautenstrauch syndrome
- Williams syndrome
Sometimes5–29%
26- 12q14microdeletion syndrome
- Autosomal dominant Robinow syndrome
- Autosomal recessive Robinow syndrome
- Axenfeld-Rieger syndrome
- Cenani-Lenz syndrome
- Cleft lip and alveolus
- Cleft lip/palate-ectodermal dysplasia syndrome
- Deafness with labyrinthine aplasia, microtia, and microdontia
and 18 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: Missing between one and six teeth
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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.