Acute rheumatic fever (ARF) and its sequela rheumatic heart disease remain significant causes of cardiovascular morbidity and mortality worldwide. When caring for patients originating from a geographic setting where ARF is endemic, a high index of suspicion for ARF is indicated. Early recognition of ARF with the initiation of treatment and secondary prevention is vital to prevent irreversible cardiac valve damage. This article covers the epidemiology, clinical manifestations, and diagnostic considerations of ARF. Specifically, the differing diagnostic criteria between high- and low-risk populations are emphasized. It will also review management and prevention strategies for ARF. [Pediatr Ann. 2022;51(12):e457-e460.].