Lipomatous melanocytic nevomatosis.

Giant congenital melanocytic nevi (GCMN) are rare and occur in about one out of every 200,000-500,000 births. Their importance resides in the aesthetic alteration they produce and in the possibility of malignant transformation or in their association with nevi in the central nervous system as a distinctive syndrome: neurocutaneous melanoblastosis or nevomatosis, due to the fact that nervous system lesions are produced by infiltration of nevus cells. We present here a patient with a combination of GCMN with an outstanding lipomatosis located on the same area.