Abiotrophia defectiva, also known as nutritionally variant streptococcus, is part of the normal flora of the oral cavity and urogenital and intestinal tracts and is a rare cause of infective endocarditis. It is fastidious or difficult to culture and associated with high rates of septic embolization, treatment failure and mortality. We describe an unusual presentation of infective endocarditis with severe mitral valve regurgitation due to Abiotrophia defectiva in an immunocompetent patient. After a complicated hospital course, surgical replacement of both the mitral and aortic valves was performed. We suggest that this patient likely had subacute infective endocarditis before diagnosis and treatment of her urinary tract infection, and following treatment failure, she developed life-threatening infective endocarditis. This case report highlights that patients with Abiotrophia defectiva infections are at high risk for infective endocarditis and that the clinical progression from this infection can be slow, with difficulty isolating the pathogen, which can significantly impact patient outcome.