Small fiber neuropathy (SFN) is common and can be associated with many medical conditions, including reports of an association with COVID-19. A Dutch study suggests a prevalence of 52.95 per 100,000 population that increases with age.1 Standardized diagnostic criteria for SFN are not fully established and skin biopsy remains the diagnostic test considered most reliable. Autonomic testing is useful when autonomic symptoms are present. Screening for associated conditions is important for etiology-specific treatment to control symptoms and slow down disease progression. The significance of new association with autoantibodies, including antibodies to trisulfated heparin disaccharide (TS-HDS) and fibroblast growth factor 3 (FGFR3), needs further investigation. Treatment should be individualized to control underlying causes and alleviate pain. Intravenous immunoglobulin (IVIG) is ineffective for treatment of idiopathic painful SFN. Progression is slow, and most people affected by SFN do not develop large fiber involvement over time. Symptoms of SFN, including painful paresthesia and dizziness, and sedative side effects of pain medications can negatively affect quality of life. Early diagnosis and individualized treatment are important for controlling SFN symptoms and optimizing daily functions. Here, we review the recent advances in the diagnosis and management of SFN.