Background: Pediatric acute-onset neuropsychiatric syndrome, further subcategorized as pediatric autoimmune neuropsychiatric disorders associated with streptococcus, is a form of idiopathic autoimmune encephalitis (IAE). Poststreptococcal autoimmunity seen in Idiopathic autoimmune encephalitis manifests as various neuropsychi-atric symptoms such as obsessive rituals, tics, anxiety, depression, and many others. Idiopathic autoimmune encephalitis has clinically heterogeneous phenotypes that make accurate diagnosing difficult, although diagnos-tic testing such as the Cunningham Panel increases the likelihood of finding effective treatments. Current recom-mended treatments include psychiatric medication, behavioral intervention, antibiotics, anti-inflammatory therapy, and immunomodulating therapy.Objective: To provide an updated review on the diagnosis, management, and treatment of pediatric autoimmune neuropsychiatric disorder associated with streptococcus and pediatric autoimmune neuropsychiatric syndrome, also referred to as IAE.Results: Information from 47 sources was used to outline current knowledge of IAE pathophysiology, clinical manifestations, and epidemiology, and to outline diagnostic recommendations and current treatment guidelines. Gaps in knowledge, in addition to current controversy, were also outlined to provide a thorough background of this condition and future needs for IAE research. Conclusion: Owing to the complexity and variability in ways patients with IAE may present to the allergist/ immunologist office, an interdisciplinary approach is imperative to provide patients with the best medical care. Still, more research is needed to further elucidate the mechanism(s) and optimal treatment algorithm for IAE to facilitate broader recognition and acceptance of this condition by the medical community.(c) 2023 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.