Aerococcus urinae is a rare cause of urinary tract infections, mainly in elderly men with underlying urinary tract pathologies. In addition, it has been described as a pathogen in balanitis, soft tissue infections, septicemia and endocarditis. To date ten cases of A. urinae endocarditis have been reported in the literature with a high rate of mortality (7/10) and morbidity, as two out of three survivors suffered from neurovascular complications. Here we present the case of an additional patient who was successfully treated with surgical valve replacement and antibiotic therapy consisting of ceftriaxone and netilmicin for 6 weeks. Furthermore, we review all reported cases of A. urinae endocarditis with emphasis on predisposing factors and therapeutic options.