Visual disturbance is not necessarily included in the common symptoms of internal carotid artery occlusive disease although it is occasionally the only clinical sign of this disease. We analyze the clinical features of the internal carotid artery occlusion and stenosis presenting visual acuity disturbance and the role of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis for ameliorating visual problems.We summarize 10 cases of internal carotid occlusive disease that presented visual disturbance as their first symptom retrospectively. Seven patients were seen by ophthalmologists initially and consulted a neurosurgeon for a second evaluation. On average, 56 weeks were required for the patients whose only symptom was visual disturbance to be diagnosed as internal carotid artery occlusive disease. All patients suffered retinal ischemia on the affected side, and secondary neovascular glaucoma was seen in most cases. All cases showed decreased cerebral blood flow on SPECT. Retrograde blood flow of the ophthalmic artery was recognized in 9 cases. STA-MCA anastomosis was performed for the patients who showed decreased cerebral blood flow and cerebral circulation reserve capacity on single photon emission computed tomography.STA-MCA anastomosis was performed in 5 patients. Improvement of visual acuity was not apparently recognized in any patients after the surgery although decrease of iridic neovasculization and improved retinal circulation was confirmed.Visual disturbance of internal carotid occlusive disease might be caused by retinal blood steal by chronic ischemic brain. STA-MCA anastomosis might be effective for such a condition because of reduction of steal flow to the brain. Early diagnosis and treatment are essential to obtain recovery of visual acuity. More coordination with ophthalmologists is essential to raise the quality of treatment.