Barlow disease represents the extreme form of the degenerative mitral valve spectrum described by Carpentier. The myxoid degeneration of the mitral valve may result in a billowing leaflet or in a prolapse and myxomatous degeneration of the mitral leaflets. There are increasing evidences of the association between Barlow disease and sudden cardiac death. It is common in young women. Symptoms include anxiety, chest pain and palpitation. In this case report, the markers of risk for sudden death such as typical ECG changes, complex ventricular ectopy, a spiked configuration of the lateral annular velocities, mitral annular disjunction and evidence of myocardial fibrosis were assessed.