Background: Left ventricle non-compaction (LVNC) or persistence of spongy myocardium is a rare form of congenital cardiomyopathy which presents with cardiac failure, thromboembolic events, arrhythmia and sudden death.
Case Presentation: We report a family with two deceased children and two alive offspring diagnosed with left ventricular (LV) non-compaction. A 9-year-old boy and a 16-year-old girl of our reported family suddenly died following exercise and emotional provocation.