Background: Small retrospective analyses suggest that end-stage renal disease (ESRD) patients do not obtain as much of a survival benefit from an implantable cardioverter-defibrillator (ICD) as non-ESRD patients do. We aimed to assess the survival effect of an ICD in ESRD patients with left ventricular dysfunction. Methods: Data from two registries identified ESRD patients with an ICD and ESRD patients with left ventricular dysfunction (defined as ejection fraction <0.35). Cox proportional hazards regression was performed, including certain predefined covariates to assess the effect of an ICD on survival. Results: Overall survival in the full cohort was a median of 4.7 years with 20 deaths in the ICD group and 29 deaths in the no-ICD group. The median survival in the ICD group was 8.0 years and 3.1 years in the no-ICD group. Crude analysis showed a better survival in the ICD group as compared to the no-ICD group (p = 0.016). The multivariable analysis confirmed that the ICD group had significantly less all-cause mortality compared to the no-ICD group (HR: 0.40; 95% CI: 0.19, 0.82; p = 0.013). Conclusion: An ICD is associated with a higher survival in ESRD patients with left ventricular dysfunction. This result merits further study in a larger cohort of patients.