Cardiac papillary fibroelastoma (PFE) is a benign cardiac tumor that mainly occurs in the valve leaflets of the left heart. The patient was a 72-year-old man diagnosed with cerebral infarction. Contrast-enhanced computed tomography and transesophageal echocardiography revealed a 12-mm mass in the left atrial appendage (LAA), and we decided to undergo emergency cardiac tumor resection due to embolism onset. Postoperative pathological examination revealed an extremely rare cardiac PFE originating in the LAA.