Infective endocarditis (IE) in chronic haemodialysis (HD) is significantly more common and causes greater morbidity and mortality than in the general population, being second only to cardiovascular disease as the leading cause of death in this group of patients. Because of the peculiarity of this group of patients, it has been recently proposed to add a fifth category (health-care associated and HD-associated IE) in the actually four categories classification of IE (namely, native valve IE, prosthetic valve IE, IE in e.v. drug users, and nosocomial IE). Given that rates of acceptance into HD are increasing (including a higher proportion of older patients in whom valvular calcification is virtually ubiquitous), and along with improved survival in HD patients, the incidence of IE in this subset of patients will probably increase with significant diagnostic and therapeutic implications. In particular cardiac, diagnostic, echocardiographic, and surgical expertises are required to correctly identify patients at higher risk and who may benefit from surgical treatment. The aim of this review is to clarify the peculiar features of chronic HD patients with regard to pathogenesis, diagnosis, current therapeutic options, and determinants of prognosis of IE.