Background: Multiple system atrophy (MSA) is a rapidly progressing neurodegenerative disorder without effective disease-modifying therapies. Because of a lack of reliable diagnostic biomarkers, there has been increasing interest in using magnetic resonance imaging (MRI) to improve the diagnostic accuracy of MSA.Methods: This review summarizes recent literatures on the role of MRI in the diagnosis of MSA. Results: Several MRI abnormalities on conventional MRI already are included in the current diagnostic criteria for MSA. Other features on conventional MRI are also used to make a diagnosis of MSA or to rule out alternative diagnoses. On the other hand, some of the MRI findings that were previously considered suggestive of a diagnosis of MSA are now being challenged, because it turned out that they were not as specific to MSA as previously thought. More advanced MRI modalities, including susceptibility-weighted imaging, diffusion-weighted imaging, diffusion tensor imaging, voxel-based morphometry, and cortical thickness analysis, are now used to study the changes in the brains of patients with MSA. Furthermore, studies have produced promising results demonstrating the use of MRI as a tool for monitoring and assessing disease progression in MSA. Conclusions: MRI is useful and indispensable in the diagnosis of MSA and also possibly for monitoring disease progression. In this regard, well-designed, long-term, prospective studies on large numbers of patients are needed.