Hypertrophic cardiomyopathy (HCM) is a heterogeneous and relatively common genetic cardiac disease that has been the subject of intense scrutiny and investigation for over 40 years. HCM is an important cause of disability and death in patients of all ages, although unexpected sudden death in the young is perhaps the most devastating component of the natural history. Therefore, while HCM is uncommon in pediatric cardiology practice, it is nevertheless a disease of great importance to young people and those clinicians charged with their care. Due to marked heterogeneity in clinical expression, natural history and prognosis, diagnostic and management strategies often represent a dilemma (and even the source of controversy) to both primary care clinicians and cardiovascular specialists. Consequently, it is timely to place perspective and clarify many of these relevant clinical issues, and profile the rapidly evolving concepts regarding HCM, especially as they may impact on this disease in childhood.