A survey was conducted on 30 unaffected individuals from a family with autosomal dominant late onset spinocerebellar ataxia in South Africa. The psychological impact of the disorder on individual lives, risk awareness, attitudes towards affected kin and reproduction were evaluated. Respondents employed various psychological strategies to deal with the threat of developing the disorder. In a comparison of "assigned" risk with "perceived" risk, 80% of unaffected persons reported incorrect perceptions of personal risk status. The disorder had little impact on attitudes concerning reproduction; the majority of individuals at risk wanted more children. These issues need to be addressed in the genetic and predictive testing service for familial ataxia in South Africa.