Objective To systematically retrieve, evaluate, and summarize the best evidence on exercise management for children with hemophilia, and to provide references for clinical practice and home care. Methods Relevant evidence regarding exercise ma-nagement in children with hemophilia was searched in Chinese and English official websites and databases from the inception of databases to November 1, 2025.Two researchers independently performed literature quality appraisal, evidence extraction and integration. Results A total of 15 articles were included.Forty pieces of best evidence were summarized across six aspects:goals and principles of exercise management, pre-exercise assessment, types and modes, frequency and intensity of exercise, safety protection and bleeding management, and health education and psychological support. Conclusion The synthesized best evidence for exercise management in children with hemophilia provides systematic references for clinical practice and home care.Further validation of the applicability of the evidence in clinical settings is needed.