Rare diseases · Sign or symptom
Renal agenesis
Absent kidney
HP:0000104
What it means
Agenesis, that is, failure of the kidney to develop during embryogenesis and development.
Renal agenesis can occur as a unilateral or bilateral trait.
Rare diseases that can present with this40
Very common80–99%
7Common30–79%
9- BNAR syndrome
- Caudal regression syndrome
- Distal 16p11.2 microdeletion syndrome
- Hirschsprung disease-deafness-polydactyly syndrome
- Kallmann syndrome-heart disease syndrome
- LUMBAR syndrome
- Microcephaly-cardiac defect-lung malsegmentation syndrome
- Syndactyly-telecanthus-anogenital and renal malformations syndrome
- VACTERL/VATER association
Sometimes5–29%
18- Branchio-oculo-facial syndrome
- Desmosterolosis
- Diamond-Blackfan anemia
- Floating-Harbor syndrome
- HNF1B-related autosomal dominant tubulointerstitial kidney disease
- Iniencephaly
- Intellectual disability-cardiac anomalies-short stature-joint laxity syndrome
- Kallmann syndrome
and 10 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 kidney · Renal aplasia
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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.