AbstractIntroductionSydenham's chorea (SC), prevalent in developing countries and occasionally affecting developed ones, poses a clinical challenge due to the lack of systematic guidelines for diagnosis and treatment. Resulting from Group A Beta‐HemolyticStreptococcusinfection, SC presents various symptoms. This review aims to collect and evaluate available data on SC management to propose a cohesive treatment plan.MethodsWe searched PubMed, the Cochrane Library, Google Scholar, and ClinicalTrials.gov for literature on SC management from inception until 24th July 2022. Studies were screened by titles and abstracts. Cochrane Collaboration's Risk of Bias tool (RoB‐1) assessed Randomized Controlled Trials, while the Risk of Bias In Non‐randomized Studies of Interventions (ROBINS‐I) tool evaluated nonrandomized studies.ResultsThe review includes 11 articles assessing 579 patients. Excluding one study with 229 patients, of the remaining 550 patients, 338 (61.5%) were females. Treatments used were dopamine antagonists in 118 patients, antiepileptics in 198, corticosteroids in 134, IVIG in 7, and PE in 8 patients. Dopamine antagonists, particularly haloperidol, were the primary treatment choice, while valproic acid (VPA) was favored among antiepileptics. Prednisolone, a corticosteroid, showed promising results with weight gain as the only side‐effect. Our review emphasizes the importance of immunomodulators in SC, contrasting previous literature.ConclusionDespite limitations, dopamine antagonists can serve as first‐line agents in SC management, followed by antiepileptics. The role of immunomodulators warrants further investigation for conclusive recommendations.