We report a case of a 48-year-old man who complained episodes of angina at rest. Instrumental investigation demonstrated a mass involving the ventricular side of the aortic valve suggestive for papillary fibroelastoma; an anomalous origin of the right coronary artery from the left sinus of Valsalva and no significant coronary artery disease were shown as well. The patient underwent open heart surgery, the tumor was excised and the aortic valve was spared. Pathological examination confirmed the diagnostic hypothesis of papillary fibroelastoma. The resolution of anginal symptoms after surgery suggests the possibility of papillary fibroelastoma causing these symptoms.