Hemodialysis patients carry a large burden of cardiovascular disease; most onerous is the high risk of sudden cardiac death. Defining sudden cardiac death among hemodialysis patients as well as understanding its pathogenesis is challenging, but inferences from existing literature reveal differences between sudden cardiac death among hemodialysis patients and the general population. Vascular calcifications and left ventricular hypertrophy may play a role in the pathophysiology of sudden cardiac death whereas traditional cardiovascular risk factors seem to have a more muted effect. Arrhythmic triggers also differ in this group as compared to the general population, with some arising uniquely from the hemodialysis procedure. Combined, these factors may alter the types of terminal arrhythmias that lead to sudden cardiac death among hemodialysis patients, having important implications for prevention strategies. This review highlights current knowledge on the epidemiology, pathophysiology, and risk factors for sudden cardiac death among hemodialysis patients. We then examine strategies for prevention including use of specific cardiac medications and device-based therapies such as implantable defibrillators. We also discuss dialysis-specific prevention strategies including minimizing exposure to low potassium and calcium dialysate, extending dialysis treatment times or adding sessions to avoid rapid ultrafiltration, and lowering dialysate temperature.