Acute rheumatic fever (ARF) is a complex disease with several clinical manifestations. Its most significant long-term complication is valvular heart damage, commonly referred to as chronic rheumatic heart disease. The risk of ARF varies globally, with over 80% of cases occurring in low- and middle-income countries, highlighting the role of socioeconomic factors. A comprehensive understanding of the risk factors associated with ARF and its clinical, genetic, and sociodemographic mediators can help clinicians identify high-risk individuals, develop effective management strategies, enhance target screening and active case-finding initiatives, and ultimately improve patient outcomes. This review aimed to provide an overview of ARF and its global burden, focusing on the established and potential risk factors associated with its development.