Nutritionally variant streptococci (NVS) are an unusual but significant cause of endocarditis, with increased morbidity and mortality compared to those caused by enterococci or viridans streptococci. For many reasons, NVS endocarditis has remained very difficult to treat medically with antibiotics, and when the bacteria infect prosthetic valves, surgical intervention has historically been required to effect cure. We report the first case of Granulicatella adiacens prosthetic valve endocarditis (PVE) in a patient with congenital heart disease who was successfully treated with a regimen of vancomycin, gentamicin, and rifampin and provide the first review of the literature on NVS PVE, as well as discussion of logistics behind the use of this antibiotic regimen.