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A cardiac papillary fibroelastoma (CPFE) is reported to be the second most common cardiac neoplasm after myxoma cordis. CPFEs are histologically benign, frequently asymptomatic, but highly thrombogenic, which could lead to systemic and peripheral embolization. We present a case of a 68-year-old-patient, with a history of angioosteohypertrophy syndrome, who presented at our emergency department (ED) with symptoms of transient ischemic attacks. A thorough investigation, including echocardiography, revealed a neoplasm on the left coronary cusp (LCC) of the aortic valve. The neoplasm was resected via a valve-sparing shave via the right anterior thoracotomy (RAT). The pathological assessment confirmed it to be CPFE. CPFE is a rare but treatable cause of thromboembolism. The removal of CPFEs has classically been performed through a full median sternotomy. We like to present the first case of a valvesparing removal of a CPFE on the aortic valve through a RAT approach.