Papillary fibroelastoma (PFE) is a generally rare benign cardiac tumor, which commonly involves left heart valves. Since having minimal effect on hemodynamics and valvular function, the majority of patients suffering from PFE are free of any symptom. However, some life-threatening complications might occur in these patients. These tumors are usually diagnosed incidentally during transthoracic echocardiography (TTE), cardiac surgery and at autopsy. The management remains undefined and controversial. The incidence and risk factors of the disease have not been reported yet. The development and popularization of echocardiography has resulted in an increase in its detection rate.