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Purpose of Review: Sudden death is a leading cause of death in industrialized nations. Sudden death in children represents a relatively small proportion of sudden death in the population, but has devastating effects on families and communities, and often attracts significant attention., Recent Findings: Several recent studies show that a portion of previously unexplained sudden death in children is due to cardiac conditions which are potentially identifiable by 'molecular autopsy' or investigation of family members. As some of these conditions that may present with sudden death can be detected by ECG, the controversial role of screening asymptomatic children will be discussed, as will the question of increased risk associated with stimulant medications. Recent pharmacologic and nonpharmacologic advancements for those with identifiable conditions at increased risk of sudden death will be reviewed., Summary: Recent developments have refined our understanding of the various causes of sudden death in children. Post-mortem genetic testing and/or investigation of family members can be fruitful in determining a cause and identifying at-risk relatives. Given the available and potential treatments for specific disorders with increased risk of sudden death, the benefit of identifying such disorders early is clear. The debate regarding universal ECG screening is social and political, as well as scientific.