This study evaluated the prevalence and risk factors for endocarditis in patients undergoing hemodialysis with central venous catheter (CVC) and to identify the microorganisms isolated from the bloodstream. This cohort study followed 94 patients with end stage renal disease (ESRD) who developed bacteremia after the insertion of a CVC for dialysis in the hospital São Paulo, UNIFESP, Brazil. They were divided into two groups: patients with endocarditis (E+) and patients without (E-). The prevalence of endocarditis was 29%. Among risk factors, the time of hospitalization and residence of the catheter were significantly higher (p < 0.001) in group E+. The mortality rate was 15%, and lethality of endocarditis was 55%. Of the 38 microorganisms isolated in group E+, 20 were S. aureus and 45% of those were MRSA. Group E+ with MRSA presented 100% mortality. High mortality and lethality in the E+ group was observed, and of the microorganisms isolated, MRSA was the most lethal. This article provides an overview of the findings of this study.