A man with sensory neuropathy had evidence of autonomic failure: abnormal pupils, hypohidrosis, esophageal dilation, diarrhea, hypotension, orthostatic hypotension, sphincter disturbance, and impotence. Functional tests revealed abnormalities of both sympathetic and parasympathetic systems, mainly postganglionic. Autopsy revealed degeneration of posterior columns, posterior nerve roots, posterior root ganglia, and peripheral nerves. Degeneration was also observed in the sympathetic trunk, vagal nerve, and myenteric plexus. Neurons in the intermediolateral columns were preserved. Progressive sensory neuropathy with dysautonomia seems to be a new disease.