Acute rheumatic fever (ARF) and poststreptococcal reactive arthritis (PSRA) represent two distinct but related poststreptococcal conditions, both arising from an autoimmune response following a Group A streptococcal infection (GAS), typically pharyngitis [1,2]. While both conditions share a common trigger, their clinical manifestations and long-term outcomes differ significantly. ARF, a systemic inflammatory disease, can affect multiple organs, most notably the heart, potentially leading to chronic rheumatic heart disease (RHD) with permanent valvular damage [3]. Historically, ARF has been a major cause of morbidity and mortality, particularly in children and young adults, and ARF remains a significant global health concern, especially in developing countries [2]. In contrast, PSRA primarily manifests as sterile arthritis, predominantly affecting the lower extremities, and generally resolves without lasting sequelae [1]. This review explores the epidemiology, clinical features, pathophysiology, diagnosis, and management of both ARF and PSRA, highlighting their distinct characteristics and emphasizing the importance of prompt diagnosis and appropriate treatment to prevent long-term complications, particularly the development of RHD. We also discuss emerging diagnostic tools and therapeutic approaches, offering insights into potential future directions for managing these poststreptococcal conditions.