Over 140 million people live at high altitude, defined as living at an altitude of 2400 m or more above sea level. Subjects living under these conditions are continuously living under hypoxic conditions and, depending on the population, various adaptations have developed. Interestingly, subjects living chronically at high altitude appear to have a decreased frequency of obesity, diabetes and coronary artery disease. However, these benefits on health are balanced by the frequent development of systemic and pulmonary hypertension. Recently, it has been recognized that subjects living at high altitude are at risk for developing high-altitude renal syndrome (HARS), which is a syndrome consisting of polycythemia, hyperuricemia, systemic hypertension and microalbuminuria, but with preserved glomerular filtration rate. More studies should be performed to characterize the mechanisms and etiology of HARS; as such studies may be of benefit not only to the high-altitude population, but also to better understanding of the renal consequences of acute and chronic hypoxia. © 2012 The Author.