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Background and purpose: Cardiovascular events are a major cause of early death in the Huntington's disease (HD) population. Dysautonomia as well as deterioration of circadian rhythms can be detected early in the disease progression and can have profound effects on cardiac health. The aim of the present study was to determine if patients with HD and pre‐manifest mutation carriers present a higher risk of cardiovascular disease than non‐mutation‐carrying controls. Methods: This was a prospective, cross‐sectional, multicentre study of 38 HD mutation carriers (23 pre‐manifest and 15 early‐stage patients) compared with 38 age‐ and gender‐matched healthy controls. Clinical and epidemiological variables, including the main haematological vascular risk factors, were recorded. Ambulatory blood‐pressure monitoring and carotid intima–media thickness (CIMT) measurement were performed to assess autonomic function and as target‐organ damage markers. Results: Most (63.2%) patients with HD (86.7% and 47.8%, respectively, of the early‐stage and pre‐manifest patients) were non‐dippers compared with 23.7% of controls (P = 0.001). CIMT values were in the 75th percentile in 46.7% and 43.5%, respectively, of the early‐stage and pre‐manifest patients, whereas none of the controls presented pathological values (P = 0.001 and P = 0.006, respectively). Nocturnal non‐dipping was significantly associated with CIMT values in patients (P = 0.002) but not in controls. Conclusions: These results suggest that higher cardiovascular risks and target‐organ damage are present even in pre‐manifest patients. Although larger studies are needed to confirm these findings, clinicians should consider these results in the cardiovascular management of patients with HD. [ABSTRACT FROM AUTHOR]