OBJECTIVES: To describe the illness perceptions and coping mechanisms of patients with Huntington's disease (HD), and to assess their role in the well-being of these individuals. DESIGN: A single group, cross-sectional study. METHODS: In 77 individuals with a clinically confirmed diagnosis of HD, illness perceptions, coping mechanisms, motor and cognitive performance, and well-being were assessed by means of questionnaire-guided interviews, and subjected to correlational analyses, t-tests and two-stage regression analyses. RESULTS: HD patients' illness perceptions were characterized by a strong illness identity, combined with beliefs about a long duration of HD, perceived negative consequences for their daily lives, and little hope for cure or improvement of their symptoms. In turn, the coping strategies that HD patients reported adopting to deal with their disease were comparable with those adopted by reference individuals dealing with everyday life stressful situations. Where the well-being of HD patients is concerned, compared with Dutch community sample, HD patients scored significantly lower on measures of physical well-being and general health. Both illness perceptions and coping mechanisms were significant predictors of patients' well-being. CONCLUSIONS: More systematic research within a health psychology framework is justified in order to assess the role of illness perceptions and coping mechanisms in the well-being of HD patients.