Takayasu arteritis (TA) is a rare, chronic non-specific vasculitis that can lead to ocular hypoperfusion. As a result, ocular ischemic syndrome (OIS), which prominently manifests as Takayasu retinopathy, may develop subsequently. Ocular manifestations mainly consist of progressive painless vision loss and amaurosis fugax in TA patients complicated with OIS. However, due to the lack of specific clinical characteristics, it is of great significance to improve the timely diagnosis aided by multimodal imaging, especially fundus fluorescein angiography. Early diagnosis of OIS is essential for reversal of ocular ischemia and better prognosis of TA patients. Management of OIS patients associated with Takayasu arteritis requires a combination of systemic and ophthalmic treatment. Therefore, the optimal individualized regiment should be determined by disease activity and progression, which are addressed by multi-disciplinary team assessment. Ophthalmologists should further understand the clinical features and the importance of regular ophthalmologic examinations during the disease course, thus to improve the overall survival and visual outcomes.