The association of Adie's syndrome and hypohidrosis is rarely reported. The authors describe a young woman's pupillotonia with reduced sweating over the right half of her trunk and her right upper extremity. Optical and electron microscopy of the skin revealed no pathological changes. Neurophysiological examination of the excitability degree of mononeurone and muscle spindle proved reduced excitability of the alpha-motoneurone. The results of the patient's analysis contribute to a better knowledge of the clinical aspects and pathogenesis of this rare manifestation of local pathology of the vegetative nervous system. Our observation joins Vanasse's whose pathogenic interpretation of this association especially stresses the role of disorder in the intercalated neurons of the spinal chord.