Although Enterococcus faecalis is a relatively common cause of infective endocarditis, it rarely causes meningitis. A case of Enterococcus faecalis endocarditis presenting as meningitis in a 74-year-old diabetic man on chronic hemodialysis is reported. A review of the literature showed that the association of enterococcal meningitis and endocarditis has rarely been reported. This clinical association may be more common than previously recognized and it is suggested that echocardiography be considered for all patients with enterococcal hematogenous meningitis in order to rule out endocarditis.