BACKGROUND The high risk of morbidity and mortality for patients on hemodialysis who are undergoing cardiac surgery is increased for those with active infective endocarditis (AIE). This retrospective observational single-center study evaluated the impact of chronic hemodialysis on the outcome of aortic valve replacement in patients with aortic AIE. METHODS Data were retrospectively collected for consecutive patients undergoing aortic valve surgery for AIE diagnosed according to modified Duke criteria between October 1994 and January 2011. Characteristics and outcomes of patients receiving preoperative chronic hemodialysis were analyzed. RESULTS Aortic valve AIE was present in 992 patients. Forty-five (4.5%) of the aortic valve AIE patients were receiving long-term hemodialysis preoperatively, 19 of whom (42.2%) had diabetes mellitus. Mean logistic EuroSCORE was 64.2% ± 32.2%. Twenty-four preoperative septic emboli were found in 15 patients. Results of microbiologic cultures were positive in 36 patients, with the major causative organisms identified as Staphylococcus aureus (n = 17) and Enterococcus faecalis (n = 10). Isolated aortic valve replacement was performed in 19 patients (42.2%), and 26 patients (57.8%) underwent concomitant procedures. The mean follow-up was 5.3 ± 5.2 years (range, 0.1 to 17.1 years). Postoperative complications occurred in 30 patients (66.7%). Nineteen patients (42.2%) died within 30 days of surgery, which in 8 patients was attributable to a cardiac cause. CONCLUSIONS In patients receiving chronic hemodialysis who undergo aortic valve replacement for acute AIE, postoperative mortality is high, especially in patients undergoing aortic root replacement or culture-negative AIE.