Cardiac papillary fibroelastoma has been associated to high levels of antiphospholipid antibodies, either primary or in the context of systemic lupus erythematosus. We present the case of a young female with several episodes of peripheral emboli. Two-dimensional echocardiography demonstrated a tumor on the anterior mitral leaflet. The mass was resected and histologically showed a papillary architecture covered by hyperplasic endocardial cells on a layer of connective tissue and a central core of collagen and elastic fibers. The immunologic study demonstrated high titers of anticardiolipin antibodies, complement consumption and positive antinuclear antibodies. The patient keep high anticardiolipin antibodies titers at follow-up but embolization has not recurred and has no symptoms.