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Abiotrophia defectiva, a nutritionally variant streptococci can cause bacteremia, brain abscess, septic arthritis and in rare cases, infective endocarditis, which accounts for 5-6% of all cases. A. defectiva is characteristically difficult to diagnose and the mortality, morbidity and complication rates are high. Here, we discuss a case of infective endocarditis caused by A. defectiva. A 62-year-old female had previously undergone prosthetic valve replacement 6 years prior to admission. She developed infective endocarditis after tooth extraction. Her endocarditis was successfully treated with antimicrobial therapy and mitral valve replacement surgery. This is the first case of infective endocarditis caused by A. defectiva reported in Korea. This case shows that A. defectiva could be considered as a causative organism of infective endocarditis in Korea.