Isolated noncompaction of the left ventricular (LV) myocardium is a rare congenital disorder resulting from an arrest of the compaction process of the myocardial trabeculae during normal embryogenesis. It is characterized by the presence of prominent LV myocardial trabeculations and deep intertrabecular recesses that are in continuity with the LV cavity. The most common clinical presentation includes progressive LV dysfunction, life-threatening tachyarrhythmias, and the risk for severe cardioembolic events. We describe a case where isolated noncompaction of the LV myocardium was diagnosed after an embolic occlusion of the superior mesenteric artery in a 40-year-old Caucasian woman.