Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension (OH). The first clinical cases were reported by Bradbury-Eggleston in 1925 [1]. It is a rare, sporadic disease of adults. Although OH is a necessary condition for the diagnosis of PAF, the autonomic dysfunction may be more generalised, leading in particular to genitourinary and intestinal dysfunction and sweating disorders. PAF is defined by consensus (1996) as a sporadic disease characterised by idiopathic neurogenic OH with signs of generalised dysautonomia, but no other neurological signs [2]. PAF belongs to the group of α synucleinopathies and is characterised by predominant peripheral deposition of α -synuclein in autonomic ganglia and nerves [3], [4], [5], [6]. However, in some cases subtle neurological signs may be associated and in a significant number of patients, this clinical presentation may also correspond to a premotor or precognitive phase of another synucleinopathy with central nervous system (CNS) involvement and may convert to Parkinson's disease (PD), multiple system atrophy (MSA), or dementia with Lewy bodies (DLB) as the disease progresses [7], [8], [9]. When faced with a clinical presentation of severe autonomic failure, it is also necessary to rule out other aetiologies, some of which may benefit from curative treatment.