Congenital Nevi Clinical Presentation

Updated: Apr 05, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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The presence of congenital nevi may be reported by adults, adolescents, or the parents of infants and children.


Physical Examination

Nevi may be located anywhere on the body. Classification as a congenital nevus depends in large part on an accurate history or photographs or medical reports from birth.

Scalp nevi in children younger than 18 years old tend to have perifollicular hypopigmentation that creates the appearance of scalloped, irregular borders if occurring on the periphery, or variegation in pigmentation, if occurring within the nevi. [25]

Congenital melanocytic nevi affecting acral volar skin in children are larger, more asymmetrical, and commalike compared with their acquired counterpart. [26]



Patients with giant congenital melanocytic nevi have an increased risk of developing melanoma (as high as 5-7% by age 60 y). The lifetime risk of malignant transformation associated with smaller nevi is surely smaller than that for giant nevi but is unknown at this time.

Large congenital melanocytic nevi are associated with an increased risk for developing cutaneous melanoma, leptomeningeal melanoma, neurocutaneous melanocytosis, malformations of the brain, and, rarely, rhabdomyosarcoma and liposarcoma. The risk of developing malignancy in association with congenital melanocytic nevi is dependent on the size of the nevus; the risk of developing neurocutaneous melanocytosis correlates best with the number of satellite nevi. [27]

Congenital melanocytic nevi may be linked with neurologic melanocytic (neuromelanocytosis, melanoma) and nonmelanocytic (syringomyelia, related hydrocephalus, ependymoma, meningioma, astrocytoma, choroid plexus papilloma, pineal germinoma, and malformations such as Dandy‐Walker and Arnold‐Chiari malformations) findings and possibly hypophosphatemic rickets. [28]