Huntington's disease is an inherited neurodegenerative disorder, the cause of which is unknown. Excitotoxicity, mitochondrial dysfunction and oxidative stress are all likely to contribute to the striatal cell death that occurs in this disorder. There are accumulating data indicating that under specific circumstances, dopamine, which occurs in high concentrations in the basal ganglia, might be neurotoxic. In this article, the current models used to study Huntington's disease are reviewed and the recent findings that implicate dopamine in the pathophysiology of this progressive disorder are discussed. Although many questions remain unanswered, the dopaminergic system could contribute to striatal vulnerability in Huntington's disease and provide a novel avenue for the development of new therapies.