Four patients with anhidrosis associated with normal sweat glands at skin biopsy are reported. One patient had a positive family history. All of them had pupillary asymmetry. Pharmacologic tests evinced evidence of parasympathetic denervation of the pupil in three and sympathetic denervation in the other patient. Autonomic function tests revealed intact cardiovascular reflexes. Restricted involvement of functionally and geographically distant population of autonomic neurons suggests their possible ontogenetic or biochemical similarities.