Objective To clarify differences in clinical characteristics and outcomes between patients with infective endocarditis (IE) receiving long-term haemodialysis (HD group) and those not receiving haemodialysis (non-HD group). Methods Medical records of patients with IE, admitted to hospital between January 2010 and December 2017, were retrospectively studied. Clinical characteristics and outcomes were compared between HD and non-HD groups. Risk factors for IE were assessed by COX regression. Results Twenty-one HD and 143 non-HD patients were included. Predisposing heart conditions were more frequently observed in the non-HD versus HD group (90.9% versus 19.0%). Inappropriate antibiotic therapy rate before admission and proportion of methicillin-resistant Staphylococcus aureus and Enterococcus-associated IE was higher in the HD versus non-HD group. In the HD group, fewer patients underwent heart surgery (9.5% versus 51.7%), all-cause in-hospital mortality was higher (52.4% versus 21%), and survival rate was lower versus the non-HD group. COX regression analysis revealed that haemodialysis, use of central venous catheter (CVC) and inappropriate antibiotic therapy before admission increased IE mortality, while surgery improved long-term prognosis. Conclusions Haemodialysis patients with IE may have higher mortality and lower survival rates than patients with IE not receiving haemodialysis. Haemodialysis, use of CVC and inappropriate antibiotic therapy before admission may increase IE mortality. Surgery may improve long-term prognosis.