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Papillary fibroelastoma, a benign cardiac tumor, is a rare clinical condition. Before the echocardiography era, the diagnosis was often occasional, especially during other cardiac procedures; but nowadays it has improved with the advent of higher-resolution imaging technology. The clinical presentation can vary from asymptomatic to severe complications such as cerebrovascular or ischemic events due to tumor embolization.We present the case of a 33-year-old female with a papillary fibroelastoma on the anterior leaflet of the mitral valve undergoing clinical examination for claudicatio intermittens. An occlusion of the left common femoral artery was present. The diagnosis was made posteriorly after embolectomy. All cardiac imaging investigations were negative. The heart team decided a strict echocardiographic follow-up every 3 months, and after the first visit a pedunculated mass was detected on the anterior mitral leaflet. The surgical management included urgent resection of the tumor on cardiopulmonary bypass using a minimally invasive approach via a right anterior minithoracotomy. The postoperative course was uneventful. We emphasize the need for surgical treatment given the potential risk of relapse. When no mass is detected on imaging, a strict echocardiographic follow-up and antiplatelet therapy are mandatory.