Sudden cardiac death (SCD) in children is defined as an unexpected death, caused by an abrupt cessation of systematic circulation of cardiac genesis, which occurs within one hour of the symptom onset. It is a rare and tragic event, with an estimated rate of 0,6 – 7,4 / 100 000 person-years, but accounts for up to 10% of all deaths in children after the first year of life. SCD is caused by a variety of rare cardiovascular diseases, like congenital heart defects, diseases of the myocardium, diseases of the coronary arteries and primary cardiac arrhythmias. Given the tragic circumstances of an unexpected death in a young, otherwise healthy person, the main focus is put on developing the methods of SCD prevention in children. Primary SCD prevention denotes detection of high-risk individuals via screening of asymptomatic children population, and is based on a detailed personal and family history, as well as a physical exam, with an addition of 12-channel ECG in some parts of the world. Screening cannot detect all children at risk, because symptoms are present in only 50% of the cases, and only 16% of them have a positive family history. A part of these children will therefore experience a sudden cardiac arrest, in which point the methods of secondary SCD prevention will be crucial. These methods include a timely and proper cardiopulmonal resuscitation and the use of automated external defibrillator. Survival expectancy after a cardiac arrest is higher in children than in adults, but still amounts to 4 -21%. The main reason for a low survival expectancy is considered to be the failure of recognition of cardiac arrest in children and the late initiation of cardiopulmonal resuscitation. Sudden cardiac death in children still remains an important health problem. Although our insight into mechanisms and circumstances of SCD is constantly improving, our methods of detecting high-risk individuals and assessing the efficacy of prevention measures are still inadequate. The main obstacles for routine screening are a lack of scientific evidences supporting the efficacy and the question of cost effectiveness. Therefore, a main measure for reducing the high mortality of these children currently presents basic life-support education of the public, as well as extending the public network of automated external defibrillators.