Rare diseases · Sign or symptom
Enlarged kidney
Large kidneys
HP:0000105
What it means
An abnormal increase in the size of the kidney.
The main causes of large kidney are hydronephrosis, polycystic disease, renal cell carcinoma, nephroblastoma/Wilms tumor (in children), and solitary cysts. If possible, a specific term should be used instead of this one. There are several ways of diagnosing enlarged kidney including intravenous urography.
Rare diseases that can present with this21
Common30–79%
8- AA amyloidosis
- Acanthosis nigricans-insulin resistance-muscle cramps-acral enlargement syndrome
- Beckwith-Wiedemann syndrome
- Congenital megacalycosis
- Donohue syndrome
- Glycogen storage disease due to glucose-6-phosphatase deficiency type Ib
- Paternal uniparental disomy of chromosome 1 syndrome
- Renal-hepatic-pancreatic dysplasia
Sometimes5–29%
11- ALG9-CDG
- Autosomal dominant polycystic kidney disease
- Hemihyperplasia-multiple lipomatosis syndrome
- H syndrome
- IgG4-related kidney disease
- Lymphoid interstitial pneumonia
- Mucopolysaccharidosis-like syndrome with congenital heart defects and hematopoietic disorders
- Multicystic dysplastic kidney
and 3 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: Large kidney · Nephromegaly · Renal enlargement
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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.