A 68 year-old woman presented with increasing dyspnoea (NYHA II) and systolic murmur at auscultation. Echocardiography showed thickened pulmonary valve leaflets, a systolic prolapsing mass provoking severe pulmonary stenosis (peak systolic pulmonary pressure: 42 mmHg), no regurgitation, minimal right ventricular dilatation but normal ventricular function. CT scan showed a dense structure extending from the right ventricular outflow tract (RVOT) up to the pulmonary bifurcation infiltrating the pulmonary valve (PV).