Multiple system atrophy (MSA) is a sporadic progressive neurodegenerative disease that is mainly categorized into Parkinsonism (MSA-P) and cerebellar (MSA-C) types. Its core clinical features include rapidly progressive autonomic failure and motor dysfunction. Currently, the etiology of MSA is unknown and involves multiple pathogenic mechanisms, therefore, treatment is limited to symptomatic management, and there is a lack of effective disease-modifying therapy (DMT) to slow down disease progression. Current research focuses on exploring diagnostic and therapeutic strategies combining traditional Chinese and western medicine, such as targeting α‍-synuclein pathology, regulating neuroinflammation, and enhancing neurotrophic support. Basic research and early clinical trials are evaluating the potential of various treatment options. This review aims to summarize the biological mechanisms of MSA and the latest research advancements, in order to facilitate clinical understanding of targeted treatment strategies for MSA.