Rheumatic heart disease (RHD) is heart valve(s) damage as a long-term complication of Acute Rheumatic Fever (ARF). The ARF is preventable and the global mortality of RHD has significantly decreased due to the treatment advances. However, with the increase of patients' survival rate, it causes more children and young people with disabilities which leads to a severe health burden in developing countries, such as Indonesia. The objective of this study is to identify the challenges in childhood RHD management in Indonesia. Data were extracted from PubMed, Google Scholar, and Science Direct by employing several keywords and various combinations relevant to the topic. The literature search extended to articles published within the last 10 years. The identification of literatures was limited to the publication in English or Indonesian language with free-full text publications which can be accessed by our institution. Studies encompassing evidence of epidemiology, management, and life quality of pediatric RHD were included. Results: Management of pediatric RHD in Indonesia is problematic. There are inadequate nationwide data of the disease magnitude, lack of subclinical RHD data due to limited resources, low adherence of secondary prophylaxis treatment, and lower quality of life among surviving patients. Conclusion: RHD in children in Indonesia remains a health concern. It is crucial to develop the management system of childhood RHD in Indonesia.