Purpose of Review <p>This review aims to detail recent research and guidelines that have changed the approach to exercise and sport participation in the pediatric hypertrophic cardiomyopathy (HCM) population.</p> Recent Findings <p>Recent studies, including the landmark LIVE-HCM cohort, demonstrated that vigorous exercise was not associated with death, implantable cardiac defibrillator (ICD) shock, resuscitated cardiac arrest, or arrhythmic syncope in patients with HCM. Furthermore, there is increasing evidence that restriction from physical activity (PA) has negative consequences for the cardiovascular health and emotional well-being of children with HCM. This has influenced guidelines that promote individualized risk assessment and shared decision-making (SDM), rather than uniform restriction, in children with HCM who wish to participate in both recreational and competitive athletics.</p> Summary <p>The relationship between PA and cardiac events in children and adults with HCM is more nuanced than once believed, and the negative consequences of uniform PA restrictions have become increasingly clear. Guided by recent prospective cohort studies, the approach to adolescents and young adults with HCM is changing, now emphasizing the role of individualized risk assessment and SDM.</p>