Rare diseases · Sign or symptom
Unilateral renal agenesis
Absent kidney on one side
HP:0000122
What it means
A unilateral form of agenesis of the kidney.
Rare diseases that can present with this35
Common30–79%
7Sometimes5–29%
18- 8q24.3microdeletion syndrome
- Cockayne syndrome
- Distal 22q11.2 microduplication syndrome
- DYRK1A-related intellectual disability syndrome due to 21q22.13q22.2 microdeletion
- Emanuel syndrome
- Houge-Janssens syndrome type 2
- Mayer-Rokitansky-Küster-Hauser syndrome
- Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
and 10 more in this range
Rare1–4%
9- 7q11.23microduplication syndrome
- Autosomal dominant cutis laxa
- Cockayne syndrome type 3
- Congenital adrenal hyperplasia due to cytochrome P450 oxidoreductase deficiency
- DYRK1A-related intellectual disability syndrome
- Intellectual disability syndrome due to a DYRK1A point mutation
- Inverted duplicated chromosome 15 syndrome
- Oligomeganephronia
and 1 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 one kidney · Single kidney · Unilateral kidney 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.