A 50-year-old male presented with left-sided flank pain. Abdominal CT showed an area of decreased perfusion involving two-thirds of the left kidney. Renal artery angiography revealed bilateral duplication of renal arteries with a total occlusion of the proximal artery on the left side. As a source of embolism, we identified a cardiac papillary fibroelastoma (PFE) attached on the non-coronary cusp of the aortic valve by using transesophageal echocardiography with concomitant moderate aortic insufficiency. Fragmentation of cardiac PFE was the most probable cause of embolism of the renal artery. To the best of our knowledge, this is the first case report describing a patient with a cardiac PFE who presented with kidney infarction.