Transcriptional dysregulation is an early event and may be an important pathological mechanism in Huntington's disease (HD). However, the exact process that leads to alterations in gene expression in HD is not clear. One potential mechanism underlying transcriptional abnormalities in HD may be epigenetic alterations which regulate gene expression without changing the DNA sequence. Previous work has demonstrated that epigenetic marks, such as DNA methylation and post-translational modifications of histone proteins, are significantly altered in HD cellular and animal models as well as HD patients. Furthermore, studies have shown a therapeutic role for histone deacetylase (HDAC) inhibitors in numerous HD …models. Here, we review a range of studies describing epigenetic changes in HD as well as several potential therapeutics that target aberrant epigenetic alterations in HD.