Annual cardiovascular mortality in patients with chronic kidney disease (CKD) is much higher than in the general population. The rate of sudden cardiac death increases as the stage of CKD increases and could be responsible for 60% of cardiac deaths in patients undergoing dialysis. In hemodialysis units treating patients with CKD, cardiac arrest occurs at a rate of seven arrests per 100,000 hemodialysis sessions. Important risk factors for sudden cardiac death in patients with CKD include hospitalization within the past 30 days, a drop of 30 mmHg in systolic blood pressure during hemodialysis, duration of life on hemodialysis, time since the previous dialysis session, and the presence of concomitant diabetes mellitus. As a result of the adverse cardiomyopathic and vasculopathic milieu in CKD, the occurrence of arrhythmias, conduction abnormalities, and sudden cardiac death could be exacerbated by electrolyte shifts, divalent ion abnormalities, diabetes, sympathetic overactivity, in addition to inflammation and perhaps iron deposition. Impaired baroreflex effectiveness and sensitivity, as well as obstructive sleep apnea, might also contribute to the risk of sudden death in CKD. The likelihood of survival following cardiac arrest is very low in dialysis patients. Primary and secondary prevention of cardiac arrest could reduce cardiovascular mortality in patients with CKD. Cardioverter-defibrillator implantation decreases the risk of sudden death in patients with CKD. The decision to implant a cardioverter-defibrillator should be influenced by the patient's age and stage of CKD.