Pure autonomic failure (PAF) is an uncommon disorder, characterized by autonomic failure without other neurological deficits. We report here an autopsy case of pure autonomic failure with pathological features of Parkinson's disease. At age 79, the patient developed slowly progressive severe orthostatic hypotension, followed by decreased sweating and constipation. On admission, his blood pressure dropped after standing (from 133/97 to 71/22 mmHg) without tachycardia. Neurological examination revealed neither parkinsonism, nor cerebellar symptoms. Supine plasma norepinephrine levels were low and intravenous norepinephrine administration showed denervation hypersensitivity. He was diagnosed as having PAF and treated by amezinium, midodrine, L-threodops, and fludrocortisone with elastic stockings but without any effects. He died of pneumonia about 2 years after the onset of the disease. Postmortem study revealed Lewy bodies in the nucleus basalis of Meynert, substantia nigra. Edinger-Westphal nucleus, locus ceruleus, and dorsal vagal nucleus. Neuronal loss was found in above-mentioned sites, except for Edinger-Westphal nucleus and intermediolateral nucleus. This is a rare and important report on a patient with PAF who satisfied the strict clinical criteria on PAF reported in 1996, and showed neuropathological findings, consistent with Parkinson's disease.