Hypertrophic cardiomyopathy in infancy is rare. The spectrum of etiology and management of hypertrophic cardiomyopathy in infancy were updated in the past several decades. Currently, it is mainly composed of endocardial fibroelastosis, Infants of diabetic mothers and infantile Pompe’s disease. Myocardial ischemia and cardiomegaly are the major signs of hypertrophic cardiomyopathy in infancy. The management strategies can be variable according to different etiologies of the lesion.