We report a case treated successfully by emergency carotid endarterectomy (CEA) for progressing stroke resulting from pseudo-occlusion of the internal carotid artery (ICA). A 67-year-old male was admitted to our hospital with dysarthria. Neurological examination on admission revealed mild left-sided motor weakness and dysarthria. Computed tomography (CT) showed cerebral infarction in the territory of the perforating artery of right middle cerebral artery (MCA). Magnetic resonance (MR) imaging indicated similar findings and cervical MR angiography revealed occlusion of right cervical ICA. Cerebral conventional angiography and CT angiography revealed pseudo-occlusion of the right ICA. ECD-single photon emission tomography (SPECT) indicated low perfusion in the territory of the right ICA. Conservative therapy was performed using free radical scavengers and antiplatelet drugs, but neurological signs deteriorated. Revascularization using CEA was therefore performed. After surgery, the patient was restless with neurological abnormalities, and trans-cranial Doppler (TCD), INVOS-3100 and MRA revealed hyperperfusion. Strict control of blood pressure under propofol anesthesia allowed effective management of hyperperfusion syndrome. After a 1-month follow-up period, the patient was discharged with only mild left hemiparesis.