OBJECTIVE This article focuses on the most prevalent forms of autonomic neuropathies, but also discusses conditions such as focal and dysfunctional syndromes (altered autonomic function in the absence of structural lesions). The goal of this review is to allow the reader to promptly recognize these disorders, identify potentially reversible or treatable causes, and implement the appropriate treatment as well as supportive care. RESULTS Secondary forms of autonomic neuropathies (eg, diabetes mellitus, amyloidosis) are much more common than primary forms, of which autoimmune ganglioneuropathies represent a major component. However, the spectrum of the latter is continuously evolving and has diagnostic and therapeutic implications. Testing modalities such as autonomic testing, serum autoimmune antibody testing, and skin biopsies are becoming more widely available. CONCLUSIONS Autonomic neuropathies are relatively common conditions, and, because of the prognostic implications as well as impact on patient quality of life, they should be promptly recognized and treated aggressively. Testing is critical as other conditions may mimic autonomic neuropathies. Treatment is symptomatic in many cases, but specific therapies are also available in selected autonomic neuropathies.