Hyperhomocysteinemia has been associated with premature peripheral vascular, cerebrovascular, and coronary artery disease. It has been associated with vascular diseases including cerebrovascular disease, particularly in subjects with significant carotid stenosis. However, hyperhomocysteinemia causing unilateral complete occlusion of whole of internal carotid, common carotid, and external carotid has been rarely reported. We report a case of an adult patient aged 35-year with complete occlusion of right common carotid, internal carotid, both intracranial and extracranial part, presented with recurrent TIAs and stroke and was found to be having hyperhomocysteinemia as the cause of this complete occlusion and showed marked clinical recovery with appropriate treatment. Prompt identification and treatment of hyperhomocysteinemia, as a vascular risk factor especially in young, is of utmost importance especially when its treatment is cost-effective and can save major disability from stroke.