Rare diseases · Sign or symptom

Congenital giant melanocytic nevus

Giant pigmented mole

HP:0005600

What it means

The giant congenital nevus is greater than 8 cm in size, pigmented and often hairy. A giant congenital nevus is smaller in infants and children, but it usually continues to grow with the child.

Between 4% and 6% of these lesions will develop into a malignant melanoma.

Rare diseases that can present with this1

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: Giant pigmented hairy nevus · Giant pigmented nevus

Congenital giant melanocytic nevus

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This 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.