Cardiovascular disease represents the major cause of death in chronic kidney disease patients accounting for about 43% of all mortality causes among hemodialysis patients. Sudden cardiac death (SCD) is one of the most frequent and dangerous clinical syndrome occurring in end stage renal disease (ESRD) patients. Hemodialysis patients present a great number of non traditional risk factors for cardiovascular disease such as left ventricular hypertrophy, coronary artery disease, rapid electrolyte shifts, QT dispersion, sympathetic hyperactivity and hyperphosphatemia. The aim of the following review is to summarize epidemiological aspects and pathophysiological pathways of SCD in CKD patients, defining prevention and treatment guidelines.