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The results of a national investigation to determine the prevalence of Huntington's chorea in South Africa are presented. A total of 481 persons who have died from or are presently suffering from the disorder have been identified. The prevalence rate of 0,1 per million in the South African Negro population is much less than the estimate of 22 per million in the White and Coloured groups. It is apparent that the great variation in the ethnic distribution of the disease is a reflection of the diverse origins of the different population groups of South Africa.