Introduction Multiple system atrophy (MSA) is a rare, severe adult onset, sporadic, progressive neurodegenerative movement disorders that are still poorly understood. It is characterized by cerebellar ataxia, autonomic disorders, and parkinsonism syndrome in various combinations. The incidence rate is 0.6 cases per 100,000 people per year. Prevalence is 4- 5 cases per 100,000 people. Despite of the presence of well-established clinical criteria for multiple system atrophy, ante-mortem diagnosis is difficult. Case We present a case of 44-year-old, male who with unsteady, wide based gait and rigidity which had developed gradually for 2 years. One year after the appearance of the first symptoms, he developed dysarthria, difficulty in swallowing, dizziness when changing position, and bladder incontinence. From neurologic examination we found orthostatic hypotension, dysmetria and dysdiadochokinesia on the both side. He met all the major criteria for possible rapid progression of MSA. In addition, brain Magnetic Resonance Imaging (MRI) showed prominent solids left and right cerebellum hemispheres and slight atrophy pons. Conclusion Our case can be classified as MSA according to the diagnostic criteria because the definitive diagnosis of MSA is only based on post-mortem pathological analysis. From this case we can learn that diagnosing MRA is quite challenging especially in early years of disease. The importance of good MRI interpretation and early finding of brain MRI abnormality can improve the accuracy of MSA diagnosis.