An 86-year-old woman who underwent placement of a prosthetic aortic valve for regurgitation 5 years previously was admitted because of spiking fever. The blood culture results were positive for gram-negative rods, which were identified as Achromobacter xylosoxidans. Approximately 4 months after being sent to the hospital, transthoracic echocardiography revealed vegetation at the prosthetic aortic valve. Ultimately, a diagnosis of A. xylosoxidans endocarditis of the prosthetic aortic valve was made. We report an extremely rare case of bacteremia associated by prosthetic valve endocarditis with A. xylosoxidans. In addition, we review 10 previously reported cases of endocarditis caused by A. xylosoxidans.