Abstract
Congenital melanocytic nevi may be common, quite extensive and even benign, but a giant congenital nevus is rare and has potential for malignant transformation. Giant nevus could occur in any part of the body; though, it is most common on the trunk and may be associated with other congenital malformations such as spina bifida, meningocele. However, there is a dearth of information on its combination with ambiguous genitalia. We therefore report a case of an 8-year-old child with a giant melanocytic nevus on the face and neck with widespread satellite nevi and ambiguous genitalia.
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