A 59-year-old woman showed chronic progressive autonomic failure consisting of orthostatic hypotension and dyshidrosis. No signs of Parkinsonism, cerebellar or peripheral neuropathy were observed. Autonomic nervous function tests and findings of biopsied sural nerve suggested that this case had both sympathetic and parasympathetic dysfunctions, mainly postganglionic. A decrease in the total peripheral resistance and no increase in the heart rate on standing seemed to be the mechanism of orthostatic hypotension in this case. We conclude that pure PAF can be recognized as a distinct clinical syndrome of systematic degeneration of the autonomic nervous system.