Congenital anomalies of the coronary arteries, which are rare causes of sudden death in infancy and childhood, are becoming increasingly recognized. The majority of cases involve abnormal origins or courses of the coronary arteries, but some have absence of one of the major vessels. Sudden death occurring in a healthy-appearing 2-month-old boy is reported. An unexpected finding at autopsy was absence of the proximal right coronary artery, emphasizing the need for meticulous examination of the coronary artery system in all cases.