Objective To summarize the clinical features and management experience of infective endocarditis (IE) in maintenance hemodialysis (MHD) patients, and to provide features for early recognition and treatment of IE.  Methods  We retrospectively analyzed 8 patients with MHD-related infective endocarditis (MHD-IE) admitted to Department of Nephrology, the First Hospital of Hebei Medical University between June 2020 and October 2024. Demographic data, clinical characteristics, treatments, and outcomes were reviewed.  Results  All patients were males, with a median age of 49.5 years (IQR 41.0～59.5) and a median dialysis vintage of 48 months (IQR 15～96). Fever (75.0%) was the most common presenting symptom, followed by dyspnea (25.0%). Blood cultures were positive in 6 patients (75.0%). All of the 4 patients with tunneled cuffed catheter-related IE underwent prompt removal of the catheters after diagnosis. Two patients received valve replacement surgery. In-hospital mortality was 62.5% (5/8). All of the 3 patients with isolated right-sided IE survived.   Conclusion  MHD-IE is associated with high mortality and nonspecific clinical presentation. Staphylococcus aureus is the major pathogen. Right-sided infective endocarditis (LSIE) has a better prognosis than left-sided infective endocarditis(LSIE). IE patients with inadequate response to medical therapy or severe complications should be promptly evaluated for the possibility of surgical intervention.