The autonomic neuropathies are a group of disorders in which autonomic nerve fibers or autonomic ganglia are selectively targeted. The availability of sensitive and reproducible measures of autonomic function has enhanced our ability to diagnose these disorders. Diabetes is the most common cause of an autonomic neuropathy in the Western world. Autonomic neuropathies also may occur due to amyloid deposition, following exposure to neurotoxins including medications, or as an autoimmune parainfectious or paraneoplastic condition. Certain antibodies (eg, anti-Hu and neuronal nicotinic acetylcholine receptor antibodies) are associated with autoimmune autonomic disorders. A number of inherited autonomic neuropathies exist, and the genetic mutations for some of these have been determined.