Hypertrophic cardiomyopathy（HCM）in children progresses faster and has a higher risk of sudden death，which is a great threat to children's life and health. Presently，echocardiography or magnetic resonance serve as auxiliary diagnosis，but these methods lack the capability to detect the disease at an early stage. Studies have found that mutations in cardiac myosin binding protein-C （cMyBP-C）gene are closely related to HCM. Exploring the relationship between cMyBP-C and HCM can provide guidance for early diagnosis and treatment of HCM in children，ultimately improving prognosis. This article presents a comprehensive overview of cMyBP-C， including its molecular structure，physiological functions，relationship with HCM，as well as the diagnostic potential of S-glutathionylated cMyBP-C，the therapeutic potential of myocardial myosin ATPase inhibitors，and the gene therapy.