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These features form a substantial contribution to disease burden in <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> patients and appear to be accompanied by a number of neuroendocrine and metabolic changes, pointing towards hypothalamic pathology as a likely underlying mechanism. Neuronal inclusion bodies of mutant huntingtin, which are hallmarks of the disease, occur throughout the hypothalamus, and indicate local mutant huntingtin expression that could interfere with hypothalamic neuropeptide production. Also, several genetic rodent models of <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> show features that could be related to hypothalamic pathology, such as weight loss and circadian rhythm disturbances. In these rodents, several hypothalamic neuropeptide populations are affected. In the present review, we summarise the changes in genetic rodent models of <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> for individual hypothalamic nuclei, compare these observations to the hypothalamic changes that occur in <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> patients, and make an inventory of the work that still needs to be done. Surprisingly, there is only limited overlap in the hypothalamic changes reported in <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> patients and genetic rodent models. At present, the only similarity between the hypothalamic alterations in <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> patients and genetic rodent models is a decrease in the number of orexin\u2010expressing neurones in the lateral hypothalamus. 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