The number of patients with end-stage renal disease (ESRD) has risen dramatically over the last decade. There are 300,000 patients in the United States with ESRD who are receiving hemodialysis (HD), and the incidence is increasing at a rate of 6% to 8% per year. Bacteremia, a prerequisite for infective endocarditis (IE), occurs at a rate of 0.7 to 1.4 episodes per 100 patient-care months. Few other medical conditions, except for chemotherapy-induced neutropenia, immunosuppression, and intravenous drug abuse, are associated with higher rates of bacteremia. IE occurs in approximately 2% to 6% of patients receiving HD. The aim of this article is to review the pathogenesis, diagnosis, current therapeutic options, and determinants of prognosis of IE in patients receiving HD.