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Since the introduction of echocardiography the diagnosis of papillary fibroelastomas (PFEs) in living patients has increased. They are second most common after myxomas representing 10% of cardiac tumors. The present case is of a patient with recurrent cerebrovascular accidents and documented protein S deficiency who continued to stroke despite adequate anticoagulation. Mitral valve PFE was suspected on echocardiography and confirmed at surgical excision. Two large studies published in the last decade describe the echocardiographic and clinical characteristics of PFEs which although are histologically benign, may present with a clinical course of devastating consequences owing to its strategic location within the cardiac structures. Echocardiography, particularly transesophageal echocardiography (TEE), provides the necessary anatomical resolution and detail to ascertain location, extent of involvement, and hemodynamic implications. Tissue diagnosis is based on characteristic histopathological features of avascular fronds lined by endothelial cells, containing varying amounts of elastin. The echocardiographic characteristics along with treatment options are reviewed.