Ahomalous origin of the left coronary artery from the pulmonary artery is a rare but severe congenital cardiopathy that often causes myocardial infraction within the first months of birth, often with congestive heart failure. We report four cases, three boys and one girl. The appearance of the first symptoms is early, usually between the second and the fifth month. The chest X-ray showed cardiomegaly. Echocardiography showed dilated and hypokinesis cardiomyopathy. In all patients the diagnosis was suspected because the standard electrocardiogram showed a constant deep Q wave in leads I and aVL. Angiography with coronarography was realized in only two cases confirming the diagnosis. The left coronary was directly reimplanted into the aorta in one child at 12 months, he has improved throughout six years. Two infants died after four and six years; one child had a spontaneous progressive recovered normal ventricular function because he had an important collateral circulation from right coronary artery. In conclusion, this rare ischemic myocardiopathy is serious because it's life threatening. The indirect signs given by the electrocardiogram and the echocardiography are of a big diagnosis aid. The prognosis depends on the precocity of the chirurgical reimplantation.