Individuals with a diagnosis of Huntington’s disease (HD) have been shown to experience various emotional, behavioural and psychosocial consequences. The current study employs Leventhal’s self-regulation model to explore the biopsychosocial factors related to psychological distress in people with HD, and further examine the relationship between illness perceptions, coping and psychological distress. Eighty-seven people diagnosed with HD completed the Illness Perceptions Questionnaire-Revised adapted for the population. Participants also completed self-report measures of coping and psychological distress. Data were also collected on clinical and demographic variables previously found to be associated with psychological distress. Hierarchical multiple regression analyses demonstrated that illness perceptions of identity, treatment control and timeline cyclical were predictors of anxiety while illness perceptions of identity and perceiving the cause to be related to chance were found to be significant positive predictors of depression. The coping strategy of seeking instrumental support also contributed to scores of depression, and self-report clinical variables of pain and role functioning related to physical difficulties predicted anxiety and depression, respectively. The findings suggest that illness perceptions play a significant role in psychological distress experienced by people with HD. Consequently, a focus on interventions which might change illness perceptions, and perhaps then reduce psychological distress, would be useful for future research.