Introduction <p>Acute rheumatic fever (ARF) and acute poststreptococcal glomerulonephritis (APSGN) are serious non-suppurative complications of group A β-hemolytic streptococcal infections. Although they are traditionally linked to distinct “rheumatogenic” and “nephritogenic” strains and have different immunopathogenesis, their simultaneous occurrence in the same patient is extremely rare and the mechanism has not been fully elucidated.</p> Case presentation <p>In this study, a 15-year-old male patient who developed ankle pain and edema of the eyelids and extremities following a history of throat infection is presented. Laboratory findings were consistent with nephritic syndrome and elevated ASO levels, and echocardiography revealed rheumatic carditis with multivalve involvement. The patient was diagnosed with concurrent APSGN and ARF, and was treated with benzathine penicillin, oral prednisolone, furosemide, and antihypertensive therapy. While kidney parameters returned to normal during follow-up, mild valvular regurgitation findings persisted.</p> Methods <p>Systematic review research was conducted in the following four databases: PubMed, Scopus, Web of Science and Google Scholar published from inception to September 15, 2025. A total of 36 cases were analyzed for clinical and laboratory characteristics, treatment protocols, and follow-up results. The presented case was evaluated comparatively with these data.</p> Study eligibility criteria <p>Studies meeting the following criteria were included: (1) full-text articles available in English, (2) case reports, case series or letters to the editor, and (3) information provided on patient demographics, clinical presentation, laboratory findings, treatment and follow-up results. Articles that did not meet these criteria or were review articles without original case data were excluded.</p> Results <p>Of the 36 cases analyzed, 86.1% were pediatric. The most common clinical findings were edema (63.8%), fever (55.5%), and hypertension (55.5%). The most common cardiac finding was mitral regurgitation (32/36, 88.8%). Corticosteroids were used in 16/36 cases (44.4%). Kidney recovery was reported in 26/27 cases with follow-up (96.2%), whereas persistent cardiac sequelae were reported in 19/27 cases (70.3%), with mitral regurgitation being the most frequent residual lesion (16/27, 59.2%).</p> Conclusion <p>The simultaneous occurrence of APSGN and ARF is a rare but clinically important entity requiring a multidisciplinary approach. While the kidney prognosis is generally favorable, cardiac sequelae frequently persist and may progress. Therefore, after acute nephritic manifestations are controlled, clinical attention should shift to long-term cardiac surveillance and secondary prevention; in patients presenting with APSGN, clinicians should maintain a low threshold for echocardiography when clinical, electrocardiographic, or systemic findings raise suspicion for concurrent rheumatic carditis.</p> Graphical Abstract <p>A higher resolution version of the Graphical abstract is available as Supplementary information.</p> <p></p>