BACKGROUND Embolic stroke of undetermined source (ESUS) represents 20-30% of ischemic strokes, with a high risk of recurrence. It usually requires an extensive diagnostic evaluation to address the potential etiologies. Coronary artery anomaly (CAA) of the left anterior descending artery (LAD) is uncommon, and it is known to be linked to myocardial complications. The association of this anomaly with ischemic strokes has not been reported yet. Here, we report on a rare case of a young patient with hypoplastic LAD complicated by an impaired ventricular function that resulted in left ventricular (LV) thrombus formation as a source of recurrent ischemic strokes. CASE REPORT A 36-year-old man had a 4-year history of recurrent strokes despite maintaining antiplatelet treatment. He had no pre-existing vascular risk factors or relevant family history. The initial stroke etiology work-up was inconclusive. A transesophageal echocardiogram showed moderate ventricular hypokinesia. A coronary angiogram was initiated, and a hypoplastic (LAD) artery anomaly was found. At first, the antiplatelet therapy was maintained. Later on, he presented with transient focal neurological symptoms indicative of a transient ischemic attack. Repeated echocardiograms detected left ventricular thrombus. Apixaban was started, with successful thrombus resolution in a one-month follow-up echocardiogram. He has not had a further recurrent ischemic event for 18 months. CONCLUSIONS This case suggests that CAAs might be considered as an associated etiology of ESUS in a young patient with recurrent cerebral events. In a clinical setting, we encourage early use of advanced cerebral and cardiac imaging modalities to accurately determine the stroke etiology, target the appropriate treatment, and prevent a further neurological sequel.