Left ventricular noncompaction (LVNC) is a rare cause of cardiomyopathy that can lead to systemic embolism and ischemic stroke. LVNC results from the arrest of ventricular myocardium compaction during embryogenesis. Multiple other cardiac complications coexist with this cardiomyopathy, and one of the potential consequences is cardioembolic events causing ischemic stroke. This condition can be underdiagnosed or misdiagnosed as hypertrophic or dilated cardiomyopathy. We report a patient who presented with recurrent embolic ischemic stroke secondary to LVNC that was overlooked on initial transthoracic echocardiographic studies. After an unremarkable laboratory workup, transesophageal echocardiogram (TEE) revealed noncompaction of the myocardium within the apex of the left ventricle, confirmed by cardiac magnetic resonance imaging (MRI). The patient was anticoagulated with warfarin and discharged to a rehabilitation facility. Increased understanding and awareness of the diagnosis, clinical manifestations, and management of LVNC are advised, particularly in patients with recurrent embolic stroke of undetermined source. Screening of all first-degree relatives with this familial condition is recommended as well, as appropriate treatment can prevent cardiac complications and sudden death.