Lewy body disease is a clinicopathological entity that includes Parkinson's disease, dementia with Lewy bodies, and pure autonomic failure with Lewy bodies, all of which are characterized by varied degrees of autonomic dysfunction. Research in animal models has shown transmission of α-synuclein from peripheral autonomic nerves to the central nervous system via the autonomic nervous system, and the autonomic nervous system plays an important role in the study of Lewy body disease. This paper outlines the pathophysiology, characteristics, and treatment of typical autonomic dysfunction in patients with Lewy body disease.