A review was made of the literature on Huntington’s disease, including the clinical neurology, recent advances in pathophysiology and genetic mechanisms and psychopathology. It can be concluded that research on the latter is scarce, although the subject is relevant because of the co-occurrence of psychiatric, neurological and genetic phenomena, which may lead to novel concepts in the understanding of brain function. So far, attempts to provide a comprehensive and pragmatic description of the psychopathology of Huntington’s disease have been disappointing, probably due to the limitations of the DSM classification system in this disorder. Future research should focus not only on this classification system, but also on neuropsychological functioning, because of the degenerative nature of the disease. Systematic and controlled studies should be performed on the treatment of psychiatric abnormalities in Huntington’s disease before any conclusions can be drawn.