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ObjectiveTo conduct the first prospective surveillance study of Sydenham’s chorea (SC) in the UK and Ireland, and to describe the current paediatric and child psychiatric service-related incidence, presentation and management of SC in children and young people aged 0–16 years.DesignSurveillance study of first presentations of SC reported by paediatricians via the British Paediatric Surveillance Unit (BPSU) and all presentations of SC reported by child and adolescent psychiatrists through the Child and Adolescent Psychiatry Surveillance System (CAPSS).ResultsOver 24 months from November 2018, 72 reports were made via BPSU, of which 43 met the surveillance case definition of being eligible cases of suspected or confirmed SC. This translates to an estimated paediatric service-related incidence rate of new SC cases of 0.16 per 100 000 children aged 0–16 per year in the UK. No reports were made via CAPSS over the 18-month reporting period, although over 75% of BPSU cases presented with emotional and/or behavioural symptoms. Almost all cases were prescribed courses of antibiotics of varying duration, and around a quarter of cases (22%) received immunomodulatory treatment.ConclusionsSC remains a rare condition in the UK and Ireland but has not disappeared. Our findings emphasise the impact that the condition can have on children’s functioning and confirm that paediatricians and child psychiatrists should remain vigilant to its presenting features, which commonly include emotional and behavioural symptoms. There is a further need for development of consensus around identification, diagnosis and management across child health settings.