Coronary disease is an uncommon cause of severe morbidity and death in the young. The great majority of lesions are congenital, and anomalous origins and courses of the coronary arteries are the most common groups of diseases in the age group. Intimal proliferation and coronary arteritis are even less common causes of death in this age population. For this review, we retrospectively studied cases of coronary cardiac death in children and adolescents, from 2004 to 2007, from a statewide medical examiner's office. There were 197 natural deaths in children ages ≤16 years and 58 deaths from sudden infant death syndrome. Of these, the largest group of deaths showed no morphologic abnormalities and was attributed to arrhythmias. The next largest group of deaths comprised complex congenital heart disease (n=13; 20%), followed by myocarditis (n=11; 17%), coronary disease (n=9; 14%), cardiomyopathy (n=8; 12%), valve disease (n=4; 6%), and cardiac fibroma (n=1). Nine coronary deaths occurred in seven boys and two girls, ranging from 14 days to 16 years of age (mean, 8 years). An anomalous course of the coronaries was seen in seven of the nine coronary deaths, and the other two casualties were examples of intimal proliferations involving the coronaries. The rare examples of coronary lesions in the young are discussed in this article.