Background: In developing countries, acute rheumatic fever is the most common cause of acquired heart disease in the paediatric and adolescent population. It is believed that vulnerability to developing acute rheumatic fever is associated with several factors such as socio-economic and living conditions. Aim: Determine the incidence and clinical characteristics of acute rheumatic fever in the Central Anatolia region of Kayseri within the last 14 years, and to make a comparison of two 7-year periods. Material and methods: We performed a retrospective analysis of 624 patients who were diagnosed with acute rheumatic fever at the Department of Pediatric Cardiology in the Medical Faculty of Erciyes University between January, 1998 and December, 2011. Results: The mean age of patients was 10.9 +/- 2.7 years. The female/male ratio was 1.4. When patients were categorised according to age groups, the largest group represented 376 patients (60.3%) aged between 10 and 14 years. The estimated incidence rate of acute rheumatic fever was 7.4/100,000 in the Central Anatolia region of Kayseri. Among the major findings, the most common included carditis at 54%, arthritis at 35%, Sydenham's chorea at 25%, and subcutaneous nodules at 0.5%, respectively. No significant difference was found between the first 7-year period and second 7-year period in distributions of age, gender, and major findings. Conclusion: Although there has been socio-economic development in Turkey in the recent years, the incidence of acute rheumatic fever is still high in the Central Anatolia region of Kayseri.