With the advent of echocardiography, diagnosis of papillary fibroelastoma in living patients has been made possible, yet papillary fibroelastoma found in the living remains a very rare cardiac tumor. We report a case of papillary fibroelastoma of the mitral valve with rheumatic mitral valve stenosis. A 68-year-old woman was referred to our hospital with a mitral valve tumor and rheumatic mitral valve stenosis. She underwent anticoagulation therapy with Warfarin for 8 years since having a cerebral embolization. Echocardiography revealed a mass attached to the mitral valve, with severe mitral valve stenosis. Electrocardiography demonstrated a chronic atrial fibrillation. Tumor excision with mitral valve replacement and maze procedure were performed. Both the surgical and histological findings depicted papillary fibroelastoma. The postoperative course was uneventful and the patient has remained symptom-free one year after surgery.