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Mitral valve prolapse (MVP) is fairly common in children, often being randomly found in asymptomatic, as well as in symptomatic patients with conjunctive tissue diseases (CTD). The authors report the case of an 11-yearold girl, diagnosed at the age of 6 with MVP and severe mitral regurgitation (MR). She had surgery at the age of seven, when the mitral valve replacement with a biological valve prosthesis was performed. Four years later, the girl was admitted for sudden onset of dyspnoea with orthopnoea, haemoptysis and generalized cyanosis. The diagnosis of acute pulmonary oedema and degenerated biological valvular prosthesis correlated with severe mitral stenosis was evoked by means of clinical and echocardiographic examinations. The evolution was favourable after the replacement of the bioprosthesis with a mechanical prosthesis.