It is a rare anomaly involving primarily the endocardial layer of left ventricle with proliferation of collagen and elastic tissue from very birth. The exact etiology is not known intrauterine viral infection with Coxsackie-B is a possibility. The infant may develop florid features of CHF from very infancy (fulminating type) or the onset, may be acute. In another group CHF develops very slowly (chronic type). The clinical features are like that of mitral regurgitation (murmur of grade 1-2/6) with ECG evidence of LVH and X-ray picture showing marked cardiomegaly. Echocardiography gives the definite diagnosis by delineating bright echoes arising from LV endocardium, global hypokinesia with evidence of MR. Management is mainly medical. CHF is treated with conventional decongestive therapy, inotropic agents, ACE inhibitors and in some cases with beta-blockers