Sudden cardiac death of a young competitive athlete is a rare but tragic event. Hypertrophic cardiomyopathy and coronary artery anomalies are the most frequent causes. Most cardiovascular abnormalities go unrecognized until the time of death owing to the lack of preceding signs or symptoms suggestive of disease. Physicians responsible for the care of athletes should be familiar with the various causes of sudden cardiac death, the physiologic adaptations seen in so-called athlete's heart, and existing cardiovascular screening guidelines. The preparticipation evaluation, although it has limitations, is the major instrument readily available for prevention of sudden cardiac death. Effort should be made to follow established consensus guidelines.