We report a case in which multiple uncommon causes of an ischemic vascular accident coexisted in the same patient. The patient was admitted with signs of acute stroke. Investigation workup revealed a left atrial tumor (myxoma) and a patent foramen ovale. Intraoperatively, transesophageal echocardiography added new information: papillary fibroelastomas were found in the aortic valve. This finding dictated a change in the surgical plan, adding resection of aortic valve masses to the planned excision of the left atrial tumor and patent foramen ovale closure. The uniqueness of this case derives from the coexistence of rare primary cardiac tumors. There are only five cases in literature of myxoma concomitant with fibroelastoma and the occurrence of multiple fibroelastoma is also extremely rare. Moreover this case emphasizes the benefit of the intraoperative use of transesophageal echocardiography to improve the diagnosis and management of cardiac surgical patients.