Background: Rheumatic heart disease remains a significant public health concern, especially among children in resource-limited settings like Ethiopia. Despite effective prevention strategies, RHD persists due to factors such as poverty and limited healthcare access. Understanding the clinical characteristics and valve lesions of rheumatic heart disease is crucial for improving diagnosis and management. Objective: This study aimed to characterize the clinical features and valve lesions in children with rheumatic heart disease admitted to a tertiary teaching hospital in Eastern Ethiopia. Methods: A hospital-based cross-sectional study was conducted from September 1, 2020, to August 31, 2021, at Hiwot Fana Comprehensive Specialized Hospital. Data were collected from medical records, clinical assessments, echocardiography reports, and laboratory tests. Descriptive statistics were used for analysis. Results: A total of 39 children with rheumatic heart disease were included, predominantly females (71.8%). Shortness of breath (53.9%) and cough (38.5%) were common presenting symptoms. Acute decompensated heart failure (ADHF) was prevalent (89.7%). Mitral regurgitation (94.9%) and aortic regurgitation (66.7%) were frequent valve lesions. Other findings included mitral stenosis (56.4%) and left atrial enlargement (86.1%). Laboratory results showed mean hemoglobin of 10.29 g/dL and mean ESR of 45 mm/hr. Conclusions: This study highlights the burden of RHD in Eastern Ethiopia, with advanced disease at presentation. Mitral and aortic valve lesions were predominant, emphasizing the need for early detection and comprehensive management strategies. Collaboration among healthcare providers and policymakers is essential to address the challenges of RHD in resource-limited settings. Keywords: Rheumatic heart disease, clinical characteristics, valve lesions, Eastern Ethiopia