During the 1920s, acute rheumatic fever (ARF) was the leading cause of mortality in children in the United States. By the 1980s, many felt ARF had all but disappeared from the US. However, although ARF and rheumatic heart disease (RHD) rates remain low in the US today, disease burden is unequal and tracks along other disparities of cardiovascular health. It is estimated that 1% to 3% of patients with untreated group A streptococcus (GAS) infection, most typically GAS pharyngitis, will develop ARF, and of these, up to 60% of cases will result in chronic RHD. This article reviews the epidemiology, pathogenesis, diagnosis, and management of ARF/RHD to increase awareness of ARF/RHD for clinicians based in the US. [Pediatr Ann. 2021;50(3):e98-e104.].