Pure autonomic failure (PAF) is a rare syndrome characterized by a primitive failure of the autonomic nervous system (ANS). We have studied a 53-year old woman suffering from PAF by using ANS provocative tests investigating the cardiovascular system (standing up test, controlled ventilation, Valsalva manoeuvre, handgrip test, cold pressor test, mental stress test, plasma catecholamine levels), tests investigating the intrinsic ocular innervation (pupil reactivity), and the bladder function (urodynamic tests). Our results have shown abnormalities of all the studied parameters and they have also confirmed that these patients need a multidisciplinary autonomic evaluation to correctly identify the syndrome.