Piebaldism is a rare disorder of defective melanocyte development and migration during embryogenesis. It is inherited in an autosomal dominant fashion. It may be associated with mutations within the KIT or SNAI2 genes. As with many genetic conditions, novel mutations have been identified within certain ethnic groups, and founder effect may be present in certain countries.

This disease presents with fixed depigmented patches of the skin (leukoderma) and hair (poliosis or white forelock), which are present at birth. Depigmented patches occur on the midline ventral surface (midforehead, chest, and abdomen) and symmetrically on the mid-extremities, but the skin on back, hands, and feet is typically spared. There are no associated retinal pigment abnormalities since this is a melanoblast migration and distribution problem, affecting only the skin. Some case reports have shown associations with Waardenburg syndrome.