Four cases of Huntington's disease in two families seen in Burkino Faso, a country in western Africa, are described. All four patients were black Africans aged 43, 42, 38 and 33 years. The clinical presentation was typical with chorea and episodes of psychiatric disorders. Molecular genetics of one family showed abnormal expansion of CAG repeats on IT15 in three patients. Separate mutations are likely to be the origin of Huntington's disease in Burkino Faso.