Patients receiving dialysis are at high risk for sudden cardiac death. Although clinical trials have shown that implantable cardioverter-defibrillators (ICDs) are effective in improving survival in a variety of populations, dialysis patients have been routinely excluded from these analyses. The purpose of this meta-analysis was to synthesize the available evidence regarding the effectiveness of ICD therapy in patients receiving dialysis. Medline, EMBASE, Web of Science, and Google Scholar were searched for pertinent studies published from 1999 to 2008. In addition, hand searches of the relevant annual scientific sessions and major scientific meetings in North America and Europe from 2000 to 2008 were performed. All clinical reports describing outcomes of ICD therapy in relation to renal function were eligible. Four investigators independently extracted the data in a standardized manner. Seven studies were identified, with a total of 2,516 patients and 89 patients receiving dialysis. Despite having ICDs, patients receiving dialysis had a 2.7-fold higher mortality compared with those not receiving dialysis. The results were similar in fixed- and random-effects models. Comparing patients receiving dialysis and those with chronic kidney disease but not receiving dialysis, there was no significant difference in mortality (risk ratio 1.62, 95% confidence interval 0.84 to 3.14). No evidence of publication bias was found. In conclusion, this meta-analysis suggests that even in those with ICDs, there is still a 2.7-fold increased mortality risk in patients who receive dialysis compared with those who do not. Beta blockers may be less cardioprotective in patients with ICDs who are on dialysis.