Noncompaction of myocardium is a rare and recently defined entity that may cause cardioembolism during childhood. We report an 18-month-old girl with noncompaction of the left ventricular myocardium presenting with fatal cardioembolic stroke. The patient had a high factor VIII level, which is known to cause an increased tendency to thromboembolic events. To our knowledge, this is the youngest case with stroke associated with noncompaction of the myocardium. Patients with noncompaction should be considered for prophylactic antithrombotic treatment to prevent mortality and morbidity owing to systemic thromboembolic events, especially if they carry additional risk factors that make them prone to hypercoagulation.