Dialysis patients have extraordinarily high mortality rates. The death rate for all US dialysis patients in 2004 was 230 per 1000 patient-years. Cardiac disease is the major cause of death in dialysis patients and accounts for 43% of all-cause mortality. In the United States Renal Data System database 62% of cardiac deaths (or 27% of all deaths) are attributable to arrhythmic mechanisms. The estimated rate of sudden cardiac death in US dialysis patients in 2002 was 7% per year. There are several plausible explanations for the special vulnerability of dialysis patients to sustaining sudden cardiac death. Obstructive coronary artery disease, coupled with diminished tolerance to myocardial ischemia (in the setting of myocardial fibrosis and left ventricular hypertrophy), rapid electrolyte shifts in hemodialysis patients, and derangements in autonomic function may all contribute to this heightened risk of sudden cardiac death. This review focuses on the epidemiology of sudden cardiac death in dialysis patients, underlying mechanisms of sudden death, and potential interventions to reduce the risk of sudden cardiac death in dialysis patients (including medical therapy and defibrillators). It is unlikely that one single therapeutic intervention will prevent sudden cardiac death in dialysis patients; but a more modest (and attainable) goal is the implementation of multiple strategies to reduce the risk of sudden cardiac death in this special high-risk population.