We aimed to address gaps in current understanding of the scope and impact of discrimination, by examining a cohort of individuals at‐risk for Huntington disease (HD), to describe the prevalence of concern for oneself and one's family in multiple domains; strategies used to mitigate discrimination; and the extent to which concerns relate to experiences. We conducted a cross‐sectional survey of 293 individuals at‐risk for HD (80% response rate); 167 respondents were genetically tested and 66 were not. Fear of discrimination was widespread (86%), particularly in the insurance, family and social settings. Approximately half of concerned individuals experienced discrimination (40–62%, depending on genetic status). Concern was associated with “keeping quiet” about one's risk of HD or “taking action to avoid” discrimination. Importantly, concern was highly distressing for some respondents (21% for oneself; 32% for relatives). Overall, concerned respondents with high education levels, who discovered their family history at a younger age, and those who were mutation‐positive were more likely to report experiences of discrimination than others who were concerned. Concerns were rarely attributed to genetic test results alone. Concern about genetic discrimination is frequent among individuals at‐risk of HD and spans many settings. It influences behavioral patterns and can result in high levels of self‐rated distress, highlighting the need for practice and policy interventions. © 2012 Wiley Periodicals, Inc.