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Background Rheumatic Heart Disease (RHD) remains one of the major causes of death and disability in developing countries. This preventable, treatable but not curable form of cardiovascular disease is needlessly killing scores of children and youth mainly due to the misunderstanding of the burden of the disease in these countries. We sought to describe the prevalence of RHD at one of the major referral cardiology clinics in Ethiopia. Methods This was a retrospective cross-sectional chart review of all patients referred for a cardiopathy at the Tikur Anbessa Referral Cardiac Clinic from June 2015 to August 2018. We excluded records of patients with a non-cardiac diagnosis and those without a clear diagnosis. A predesigned and tested EXCEL form was used to collect the data. The data was encoded directly from the patient record files. MATLAB’s statistics toolbox (MATLAB2019b) was used for statistical analysis. Results Among the total 7576 records analyzed 59.5% of the patients were women. 83.1% of the data belonged to adult patients with the largest concentration reported in the 18 to 27 age group. 69.7% of the patients were from urban areas. The median age of the study population was 30 (interquartile range = 21–50). 4151 cases were caused by RHD which showed that RHD constituted 54.8% of the cases. The median age for RHD patients was 25 (interquartile range = 19–34). The second most prevalent disease was hypertensive heart disease which constituted 13.6% that was followed by congenital heart disease with 9% prevalence rate. Conclusion The results of this study indicated the extent of the RHD prevalence in Ethiopia’s cardiac hospital was 54.8%. What was more critical was that almost 70% of the RHD patients were mainly the working-age group(19 to 34 years).