Double-lumen carotid plaque is a rare pathological condition, and only few reports about this condition have been recorded in the literature. However, no study has used endovascular therapy (EVT) for the treatment of double-lumen carotid plaque. Herein, we present a unique case of double-lumen carotid plaque associated with severe stenosis that was successfully treated with staged angioplasty (SAP). Moreover, a literature review of its pathology and other treatment options has been conducted. SAP is a two-stage carotid artery stenting (CAS) that can prevent hyperperfusion syndrome after revascularization. In this study, a 62-year-old man developed walking disturbance and left hemiparesis. Magnetic resonance imaging (MRI) revealed ischemic lesions in the watershed area of the right hemisphere and an irregular plaque in the right cervical internal carotid artery (ICA). Ultrasonography showed 84% stenosis in the area and a double lumen distal to the stenosis in the right ICA. Digital subtraction angiography (DSA) revealed a double-lumen plaque with 70% stenosis based on the North American Symptomatic Carotid Endarterectomy Trial criteria. SAP was performed after medication therapy and rehabilitation, and the surgery was uneventful. A double-lumen carotid plaque associated with severe stenosis is a rare condition with a high risk of emboli and stroke. In an unstable lesion, carotid endarterectomy is the first option. However, since the patient in this case was at high risk for general anesthesia, SAP was performed. Hence, if an appropriate device is used, EVT can be a safe treatment strategy for unstable and atypical plaques as in this case.