Infective endocarditis has been a subtle and very often lethal complication of hemodialysis. Thirty-five episodes have been described to date. Antecedent infections, particularly those involving the access site, access manipulation, and dental work appear to predispose to IE. Once IE is acquired, factors associated with mortality are involvement of two or more valves, infection caused by enterococci, antecedent infection, steroid therapy, infection in the first year post-access insertion and patient age over 46. The incidence of access infection with the arteriovenous fistula is significantly less than that associated with the arteriovenous cannula. Staphylococci are the most common organisms in access infections and in IE. Gram-negative bacilli and particularly Pseudomonas aeruginosa are a frequent cause of access infection but an unusual cause of IE. Access removal may be madatory in the successful management of IE in patients on hemodialysis.