| Summary: | We report the case of a girl of 2 months of age with achromic maculesin face, trunk and extremities and white forelock frontal from thebirth that corresponds to Piebaldism. Piebaldism is a skin diseaseautosomal dominant clinically characterized by white forelock andpatches of depigmentation due to congenital absence of melanocytesin affected areas by mutations of the KIT proto-oncogene,which affects the differentiation and migration of melanoblastos. Itcourse is permanent and static, that is, new lesions don´t appearand occasionally arise islands of hyperpigmentation within the leukodermal patches. Although patients with piebaldism are generallyotherwise healthy, this disease may be socially disabling and thetreatment constitutes a true challenge sometimes with little satisfactory results being the new surgical techniques (with micrografts of autólogos melanocytes cultivated) seem to be more promisingin repigmenting localized piebald lesions
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