Nevus depigmentosus, a disorder of hypopigmentation, occurs in both sexes and all races. It most commonly presents in early infancy and childhood as a nonprogressive hypomelanotic macule. It is considered a form of cutaneous mosaicism due to somatic mutation in pigmentary genes, which results in functional impairment of melanocytes. Clinical forms include localized, segmental, and systemized. Rare cases of nevus depigmentosus may be associated with systemic features. Treatment is usually not required, although certain techniques such as suction-blister grafting, excimer laser, and cosmetic camouflage have been tried with variable results. Counseling of parents plays a significant role to allay apprehension and anxiety.