Over the past decade, CRISPR/Cas9, an advanced gene-editing technology, has emerged as a revolutionary therapeutic tool that has the potential to treat various incurable gene-altering diseases such as Huntington's disease, Cystic fibrosis, Alzheimer’s, Tay Sachs, and Duchenne muscular dystrophy. CRISPR/Cas9 is a promising potential intervention that directly modifies the targeted mutant genome in a desired way. Huntington’s disease (HD) is a neurodegenerative disease that is both hereditary and fatal in nature. It is caused by the overlong stretch of repeated CAG trinucleotides on chromosome 4 and is characterized by the degeneration of the striatum nerve cells, resulting in various cognitive, psychiatric, and motor symptoms. Studies associated with the CRISPR/Cas9 system for HD demonstrated remarkable results. Permanent inactivation of the mutant huntingtin (mHTT) allele without affecting normal HTT gene activity was observed in neural progenitor iPSC studies. Moreover, the reduction in Htt protein aggregates in the treated HD iPSCs and transgenic mouse models offered a new glimmer of hope. CRISPR/Cas9 interventions led to an improved lifespan in the in-vivo models. Although precise, there remain some challenges that need to be considered while using the CRISPR/Cas9 system, such as off-target cleavage and frameshift mutations near the target sites. Here, the CRISPR/Cas9 system is discussed. This review is intended to provide an overview of the recent advancements in the CRISPR/Cas9 system for the treatment of Huntington’s disease and the challenges associated with it. Furthermore, we summarize the studies conducted on various iPSCs and transgenic HD mouse models to help researchers find a potential treatment for HD.