Papillary fibroelastomas are rare benign tumors of the heart with predisposition for cardiac valvular involvement that were traditionally incidental findings at autopsy. Echocardiography now is allowing clinicians to diagnose these tumors in living patients. We reviewed the literature and, to our knowledge, are reporting the fifth documented case of a mitral valve papillary fibroelastoma in a living patient. Our patient was seen with bilateral transient ischemic attacks and was found to have a mitral valve tumor by two-dimensional echocardiography and cardiac catheterization. The tumor involved the entire mitral valve and subvalvular structures. The patient's valve was replaced with a bioprosthesis, and she remains free from symptoms.