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Noncompaction of the ventricular myocardium is an embryonic cardiomyopathy that is increasingly being recognized. Noncompaction of LV myocardium, right ventricular myocardium, or both can occur in isolation, in congenital heart diseases, in valvular heart diseases, in neuromuscular disorders, skeletal abnormalities and in endocrinologic abnormalities. Clinical manifestations of ventricular non-compaction include congestive heart failure, arrhythmia, sudden cardiac death and embolic events. This report is illustrative of non-compaction left ventricle associated with WPW syndrome in a 12-year-old girl presented with aborted sudden cardiac death and heart failure.