Myocardial infarction (MI) is a clinical condition that develops associated with a sudden reduction or interruption of the blood flow of the vessels supplying the heart for various reasons. The electrocardiographic, echocardiographic and enzymatic diagnostic criteria of MI have been well defined in adults, in children there are some difficulties. Although seen more often in the presence of congenital heart disease (CHD), MI may also be seen in patients without CHD. Unlike atherosclerotic coronary artery disease in adult patients, ischaemia and infarct in children are often associated with coronary artery anomalies and CHD. In addition, congenital prothrombotic diseases, vasculitis, surgical or interventional procedures may also cause ischaemia and infarct. Subendocardial ischaemia, especially aortic stenosis characterised by hypertrophy in the left ventricle is often seen in hypertrophic cardiomyopathy or hypertensive patients. The most important risk factors in neonates and infants are the presence of CHD, coronary artery anomalies and perinatal asfixia. The most frequently seen causes of pediatric myocardial infarction (PMI) are abnormal left coronary artery originating from the pulmonary artery (ALCAPA) and Kawasaki disease. Another often seen cause of PMI is patients who underwent arterial switch operations.