Hypertrophic cardiomyopathy in infancy is rare. The spectrum of aetiology 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. This study shows that myocardial ischaemia and cardiomegaly are the major signs of hypertrophic cardiomyopathy in infancy. The management strategies can be variable according to different etiologies of the lesion.