Spinocerebellar ataxia type 3（ SCA3）， also known as Machado-Joseph disease， is a neurodegenerative disorder caused by an amplification of the polyglutamine repeat sequence in exon 10 of the ataxin-3 gene（ ATXN3）. It is a rare disease of autosomal dominant spinocerebellar ataxia. Its clinical manifestations primarily include ataxia， abnormal eye movements， dysarthria， and pyramidal and extrapyramidal symptoms， accompanied by psychological disorders. In recent years， with the advancement and improvement of in genetic testing technology， the number of confirmed cases of SCA3 has been steadily increasing. However， current treatment for this disease mainly focuses on alleviating clinical symptoms. This paper briefly reviews the pathophysiological mechanisms， pathogenesis， and clinical characteristics of SCA3 in recent years， with a focus on the latest advances in gene therapy， with the aim of providing a theoretical foundation for further research on gene therapy. With ongoing research into the clustered regularly interspaced short palindromic repeats and associated protein 9（ CRISPR/Cas9） system， antisense oligonucleotides（ ASOs）， and RNA interference（ RNAi） therapies， gene editing therapy is expected to emerge as a highly effective therapeutic strategy.