Infective endocarditis caused by Abiotrophia defectiva is rarely encountered. A 67-year-old male transferred from a local hospital presented with severe dyspnea and pulmonary edema. Preoperative transthoracic echocardiography revealed severe mitral regurgitation with large vegetation. Blood cultures grew , a gram positive, nutritionally deficient streptococcus variant. Emergent mitral valve replacement through right thoracotomy was performed, and after completing six weeks of antibiotic combination therapy (vancomycin, ampicillin, and gentamicin), the patient recovered fully. Because of the need for prompt surgical treatment and long-term antibiotic therapy and lack of laboratory experience with the organism, physicians and laboratory workers should pay close attention to the possibility of A. defectiva infective endocarditis when gram positive cocci are detected in blood cultures. (Ann Clin Microbiol 2019;22:23-27)