Global biodiversity loss is disproportionately rapid on islands, where invasive species are a major driver of extinctions. To inform conservation planning aimed at preventing extinctions, we identify the distribution and biogeographic patterns of highly threatened terrestrial vertebrates (classified by the International Union for Conservation of Nature) and invasive vertebrates on ~465,000 islands worldwide by conducting a comprehensive literature review and interviews with more than 500 experts. We found that 1189 highly threatened vertebrate species (319 amphibians, 282 reptiles, 296 birds, and 292 mammals) breed on 1288 islands. These taxa represent only 5% of Earth's terrestrial vertebrates and 41% of all highly threatened terrestrial vertebrates, which occur in <1% of islands worldwide. Information about invasive vertebrates was available for 1030 islands (80% of islands with highly threatened vertebrates). Invasive vertebrates were absent from 24% of these islands, where biosecurity to prevent invasions is a critical management tool. On the 76% of islands where invasive vertebrates were present, management could benefit 39% of Earth's highly threatened vertebrates. Invasive mammals occurred in 97% of these islands, with Rattus sp. as the most common invasive vertebrate (78%; 609 islands). Our results provide an important baseline for identifying islands for invasive species eradication and other island conservation actions that reduce biodiversity loss.