Albinism represents a group of inherited abnormalities of the melanin pigment system in which the synthesis of melanin is absent or reduced, generalized (oculocutaneous albinism) or localised (ocular albinism). Recent molecular studies provide insight into the pathophysiological processes of pigmentation regulation and help our understanding of the genetic heterogeneity of human albinism. It rarely affects Europeans, frequently Africans, only a minority of Amerindians, who nevertheless, when an ethnic group is concerned, presents one of the highest incidence in the world. Historically, the African albinos were used as an alibi by the European theologians to support Adam's descent of humanity and by naturalists to affirm the alleged superiority of the white men. Anthropological data are mainly issued from Amerindians with contradictories attitudes towards albinos: both acceptance and rejection. Only the Kuna of Panama have given albinos a major place in their mythology, although in reality they frequently reject them.