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Cardiac papillary fibroelastomas (CPFE) are exceptional primary benign cardiac tumours affecting the heart valves. We report here the case of a 15-year-old boy in whom echocardiography performed for non-specific chest pain during follow-up for bicuspid aortic valve showed as accidental finding the presence of a round mobile mass without stalk attached on the inferior side of the aortic valve. The mass did not cause any outflow tract obstruction or aortic insufficiency. Electrocardiogram-gated cardiac computed tomography and magnetic resonance imaging allowed to suspect CPFE. Although the patient was asymptomatic, open cardiac surgery with elective surgical resection of the tumour was performed to avoid systematic emboli. Histology confirmed the diagnosis of CPFE. This is an exceptional case of acquired CPFE in a young patient with bicuspid aortic valve. Due to the risk of systemic embolization, aortic or coronary ostium obstruction, elective excision of such lesions is recommended.