Summary

Giant congenital melanocytic nevus is a melanocytic lesion present at birth that reaches a diameter greater than 20 cm and occurs in 1 per 500,000 newborns. Melanocytes are found in utero at about 40 days of gestation and it is thought that congenital nevi develop between the 2nd and 6th month of gestation. The orbital region is a rare site of occurrence of congenital hairy melanocytic nevus, especially the giant form. We present a rare case of a giant congenital nevus that involves the head and neck with congenital unilateral anophthalmia in a one day old girl, coming from an uninvestigated pregnancy from a 25 year old multipara woman.