High altitude retinopathy(HAR)is the pathological changes of retina and optic caused by hypobaric hypoxia when the body is in high altitude environment, and HAR is the major cause of visual loss for the plainsmen who went to high attitude area. With the development of economy and tourism of plateau regions, the prevalence of HAR has recently been increasing for more and more people going to high altitude areas. Currently, it is reported that the pathogenesis of HAR involves a host of mechanisms, including the integrity damage of blood-retinal barrier(BRB), retinal neuron injury, inflammatory mediators and cytokines produced excessively, retinal blood vessels regulate dysfunction, and hemodynamics abnormality, and the synergistic action between them. In this review, we report the findings from current studies on the pathogenesis of HAR, contributing to further studies and diagnosis and treatment.