Although it is generally recognized that pure autonomic failure (PAF) is a progressive neurodegenerative disease selectively involving the autonomic nervous system, its mode of progression remains to be settled. A 57-year-old man presented with sweat reduction on the left side during previous 3 years. The thermoregulatory sweat test revealed left-sided multi-segmental hypohidrosis more markedly on the face. Pharmacological sweating was relatively preserved except for the face. During the subsequent 8 years, he developed erectile dysfunction and overt orthostatic hypotension. Plasma norepinephrine was markedly reduced without reactive increase during the tilt-table test. The heart to mediastinum ratio in 123I-meta-iodobenzylguanidine cardiac scintigraphy was reduced. Over the following 3 years, he showed progressive and generalized postganglionic sudomotor impairment without cognitive impairment or somatic nervous dysfunctions. Present observations suggest that in some patients with PAF, pathological process might start mainly at the central level and later extends to the peripheral level.