Many scientific reports emphasized that the extent of prevalence and severity of dental fluorosis is higher among the people residing at high altitude regions than lower altitudes, even though they are exposed to drinking water with similar or less fluoride levels. This review reports various factors which enhance the risk of prevalence and severity of dental fluorosis at high altitude regions through different pathways. Especially, this review focused on ecological, physiological, biochemical and dietary factors as well as to identify potential key areas to implement further research on control of dental fluorosis risk at high altitude regions. In addition, by considering the pathways of risks, suitable remedial measures are also recommended in this review to manage the dental fluorosis risk at higher altitude regions. © 2020 Elsevier B.V.