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An 8-year-old boy suffered a cardiac arrest while playing soccer. In contrast to a similar event at the age of 5 years, resuscitation was not successful. At autopsy, the cardiac findings were of a myxomatous transformation of the mitral valve with lacerations of the posterior cusp and the left vestibular endocardium and left ventricular hypertrophy. Sudden death due to arrhythmias in association with a myxomatous mitral valve is a rare event with only about 100 cases published world-wide. To our knowledge, the present case probably reports the youngest affected individual who died of this pathological condition.