Cardiac papillary fibroelastoma is a rare, benign tumour, arising from the valvular endocardium, which could lead to life-threatening complications as myocardial ischemia. We report a case of a 54-year-old male patient who presented in the emergency room with an acute coronary syndrome with transient ST segment elevation. After ruling out coronary artery disease by coronarography, we established the diagnosis of papillary fibroelastoma by performing echocardiogram completed by computed tomography angiography. The reversible acute coronary syndrome has been caused by the prolapse of pedunculated coronary cusp tumour into the main left coronary ostium. The patient was scheduled for emergent surgery. The surgical management included a complete resection of the tumour sparing the aortic valve. The patient recovered well. A papillary fibroelastoma of the aortic valve can be revealed by an acute coronary syndrome with transient ST segment elevation. More investigations must be done to eliminate such diagnosis in the case of a normal coronarography.