Papillary fibroelastoma (PFE) is a benign tumor accounting for approximately 8% of cardiac tumors. We report a 64-year-old woman with pulmonary valve PFE associated with atrial septal defect. It was detected by a transesophageal echocardiography as a fluttering mass clinging to the pulmonary valve, and was simply removed concomitantly with a patch closure of atrial septal defect. In a review of the past literature, 43 surgical cases of PFE have been reported in Japan, and aortic valve, mitral valve and left ventricle PFE was commonly encountered in 81%. Pulmonary PFE is very rare, and only one case has been reported apart from the present one.