An 11-year-old boy developed occlusion of the left internal carotid artery (ICA) following surgical correction of atypical coarctation of the aorta. The patient was admitted to our hospital after presenting with severe hypertension secondary to abdominal aortic hypoplasia and renal artery stenosis. Reconstruction of the abdominal aorta with bypass grafting was performed without complication. However, in the postoperative period, the patient experienced recurrent transient ischemic attacks manifesting as paresthesia of the right upper limbs, dysarthria, and right-sided weakness. Cerebral angiography revealed occlusion of the C₂ portion of the left ICA and decreased resting cerebral blood flow in the left hemisphere. Extracranial-intracranial arterial bypass was performed, and the patient suffered no further adverse neurological events. Coarctation of the aorta is an uncommon congenital condition that may result in cerebral ischemic disease. The cerebrovascular circulation should be evaluated, even in patients without a pre-existing history of cerebral ischemic symptoms.