AbstractThe clinical phenotype of amyloid neuropathy is not uniform. Especially in patients with hereditary amyloid neuropathy, the age at onset and severity of autonomic neuropathy are strongly influenced by the patient’s family history and the mutant gene of transthyretin. In the past 30 years, liver transplantation has been actively performed in the hereditary amyloid neuropathy patients with an early onset, resulting in much longer survival with this disease. Furthermore, new information on the pathogeneses of transthyretin‐derived amyloidosis has been accumulating via the analysis of tissue samples biopsied or autopsied from post‐transplanted hereditary amyloid neuropathy patients. The recent introduction of non‐invasive drug therapies has given rise to the possibility of treating amyloid neuropathy. The concept of amyloid neuropathy, including autonomic dysfunction, has changed. This review discusses the clinical picture, pathology, diagnosis, therapy, and post‐treatment changes of amyloid deposition in patients with amyloid neuropathy and autonomic neuropathy.