BACKGROUND The study was carried out in Riyadh City Hospital to determine the hospital prevalence of acute rheumatic fever (ARF), its characteristics and to determine the proportion of the ARF population that have recurrent attacks. METHODS The study was an analysis of 83 children with ARF, admitted to the Children's Hospital, Riyadh, over a 10-year period (1994-2003). The diagnosis of ARF was based on clinical features as defined in the modified Jones criteria with evidence of recent streptococcal infection. The diagnosis of recurrence of rheumatic fever in children with rheumatic heart disease was based on the presence of one major criterion apart from carditis or two minor criteria, in addition to evidence of preceding streptococcal infection. RESULTS The mean age at presentation was 9 years. In 31 (37%) cases, arthritis was the only major Jones criterion. In 30 (36%) others, arthritis was associated with carditis and in 3 (4%), with chorea. Cardiac involvement was documented in 44 (53%) cases; it occurred alone in 5 (6%), with arthritis in 30 (36%), and with chorea in 9 (11%) others. Among the 44 with carditis, the pattern of cardiac involvement was valvular only (mild carditis) in 30 (68%), while it was severe in the remaining 14 (32%) cases who also had heart failure. The involvement of the mitral valve alone occurred in 26 (59%) cases in the form mitral regurgitation, while both aortic and mitral valve regurgitation were present in 11 (25%) cases, and aortic valve regurgitation alone in four (9%) others. Chorea was the only major criterion of ARF in 5 children (6%), while it occurred in association with other major criteria in 12 (15%) others. Nineteen (23%) children had recurrent attacks of ARF. CONCLUSIONS ARF continues to occur in Saudi Arabia in the period (1994-2003), despite the progress made in the socio-economic development of the country, and this is often associated with severe cardiac involvement.