Extremely old trees have important roles in providing insights about historical climatic events and supporting cultural values, yet there has been limited work on their global distribution and conservation. We extracted information on 197,855 tree cores from 4854 sites and combined it with other tree age (e.g., the OLDLIST) data from a further 156 sites to determine the age of the world’s oldest trees and quantify the factors influencing their global distribution. We found that extremely old trees >1000 years were rare. Among 30 individual trees that exceeded 2000 years old, 27 occurred in high mountains. We modeled maximum tree age with climatic, soil topographic, and anthropogenic variables, and our regression models demonstrated that elevation, human population density, soil carbon content, and mean annual temperature were key determinants of the distribution of the world’s oldest trees. Specifically, our model predicted that many of the oldest trees will occur in high-elevation, cold, and arid mountains with limited human disturbance. This pattern was markedly different from that of the tallest trees, which were more likely to occur in relatively more mesic and productive locations. Global warming and expansion of human activities may induce rapid population declines of extremely old trees. New strategies, including targeted establishment of conservation reserves in remote regions, especially those in western parts of China and the United States, are required to protect these trees.