Papillary fibroelastoma, a rare primary benign tumor, can be found anywhere in the heart, but most commonly involves the cardiac valves. Most papillary fibroelastomas do not cause symptoms and are usually incidental found by routine eehocardiography or at autopsy. However, with the advent of echocardiography, more and more patients are diagnosed in life. Early diagnosis of this condition becomes very important since it represents a surgically correctable cause for systemic emboli, stroke, myocardial infarction, and sudden cardiac death. The echocardiographic findings should be confirmed by histology because the clinical differential diagnoses includes myxoma, vegetation, thrombi, lipoma, and pseudopapillary fibroelastoma. We reported a ease of papillary fibroelastoma of the aortic valve and presented a comprehensive review of the literature.