OBJECTIVE The aim of this study was to elucidate the characteristics of chronic hemodialysis (HD) patients requiring surgery during the active phase of infective endocarditis (IE). METHODS From December 2004 to July 2015, 58 patients underwent surgery in our institute for active IE. Seven patients had been on HD for 1-15 years. Their preoperative profiles and surgical outcomes were compared to those of the other 51 patients (non-HD group). RESULTS The predominant causative microorganisms in the HD group were Staphylococcus spp, particularly methicillin-resistant Staphylococcus aureus (MRSA), whereas Streptococcus spp were predominant in the non-HD group. Prosthetic dysfunction (stuck valve after mechanical and structural valve dysfunction following bioprosthetic valve replacement), complete atrioventricular (AV) block, and annular abscess formation were more frequent in the HD group. In-hospital mortality was higher in the HD group (29% vs. 6%, p = 0.044). Actuarial survival in the HD and non-HD groups was 43% vs. 87% at 5 years and 43% vs. 76% at 10 years (p = 0.007). CONCLUSIONS Early and long term outcomes in patients with chronic HD were poor. Compared to other patients, chronic HD patients undergoing valve surgery during active IE had higher incidences of MRSA infection, annular abscess formation, postoperative valve dysfunction, and postoperative complete AV block.