We present a 60-year-old female with a 23-year history of anhidrosis with concomitant heat intolerance. At examination, we found a right-sided tonic pupil, absent tendon reflexes, and a segmental patch of compensatory hyperhidrosis in the left lower quadrant of her trunk. To further confirm this finding, a minor test (a starch-iodine test, which is used to evaluate the sudomotor function, sweating) was performed. Nerve conduction studies revealed evidence of a mild distal sensory polyneuropathy of the axonal type. Tilt-table testing showed signs of orthostatic hypotension with evidence of reduced sympathetic function. Skin biopsy excluded pathology in the sweat glands. Our patient met the criteria for a diagnosis of Ross syndrome. This case describes the long-term outcome of this rare entity and highlights how careful examination and bedside autonomic testing can confirm the diagnosis of autonomic neurological disorders.