Segmental neurofibromatosis in association with a large congenital nevus and malignant melanoma.

We describe a patient with segmental neurofibromatosis and a large congenital nevus who developed malignant melanoma in the same anatomic distribution. The overlapping presentation suggests a common etiologic link between neurofibromatosis and large congenital nevi and the possibility that neurofibromatosis increases the risk of melanoma with large congenital nevi.