Oligodontia

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Oligodontia

ORPHA:99798Morphological anomaly

Also called Selective tooth agenesis

What it is

Oligodontia is a rare developmental dental anomaly in humans characterized by the absence of six or more teeth.

Key facts

Age of onset
Childhood
Inheritance
Autosomal dominant, Autosomal recessive, X-linked recessive
Classified as
Morphological anomaly

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

EDADisease-causing germline mutation(s)
EDARADDDisease-causing germline mutation(s)
FGFR1Disease-causing germline mutation(s)
GREM2Disease-causing germline mutation(s)
IRF6Disease-causing germline mutation(s)
LRP6Disease-causing germline mutation(s) (loss of function)
MSX1Disease-causing germline mutation(s)
PAX9Disease-causing germline mutation(s)
TGFADisease-causing germline mutation(s)
WNT10ADisease-causing germline mutation(s)
WNT10BDisease-causing germline mutation(s)
SUMO1Candidate gene tested

ICD-10 codes

K00.0filed under a broader ICD-10 category — shared with 1 other rare disease

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MEDDRA 10088239MONDO 0005486MONDO 5486OMIM 106600OMIM 150400OMIM 313500OMIM 604625OMIM 610926OMIM 616724OMIM 617073OMIM 617275OMIM 620173UMLS C4082304

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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