Background/Aim. Mulstiple system atrophy (MSA) is a neurodegenerative central nervous system disorder, characterized by any combination of extrapyramidal, cerebellar, pyramidal or autonomic disturbance. The aims of our study were to define clinical characteristics of MSA patients in our population, to account for neuroradiological and electrophysiological profile of the disease and to evaluate one-dose levodopa response. Methods. We have diagnosed 29 patients as MSA, with disease duration from the first symptom 5 years on average on examination. The examinating procedure included an anamnesis and complete neurological investigations, as well as neurootological and neuroradiological examinations. The study included the patients of the Institute of Neurology of the Clinical Center of Serbia in the period of 1996-2001, who completed both clinical and diagnostic criteria for a possible and probable MSA. Results. Autonomic disturbances were documented in 93.1%, whereas extrapyramidal symptoms were seen in additional 89.3% with symmetrical onset in 60%. Levodopa response was poor or moderate. Cerebellar signs were present in 63%, while pyramidal signs occured in 78.7%. There was no a cognitive deterioration (MMSE > 24). CT scan and MRI showed cerebellar and brainstem atrophy, as well as diffuse cortical atrophy. Conclusion. Failure of additional diagnostic procedures to distinguish MSA patients required a precise understanding of their clinical specificities. Our results support this statement.