There are approximately 126,000 valid described freshwater species, comprising around 9.5% of described species diversity, many of which are key economic and nutritional resources for people globally. Freshwater ecosystems are often cited as being the most threatened of all systems. However, information on the conservation status and distribution of freshwater species has been extremely limited until recently. Over the last 10 years, the International Union for Conservation of Nature (IUCN) Global Species Programme Freshwater Biodiversity Unit has made significant progress to fill this information gap. From a base of only 1,422 species assessed on The IUCN Red List of Threatened SpeciesTM (Red List) in 2002, there are now 23,291 completed species assessments for freshwater birds, amphibians, mammals, reptiles, crustaceans, plants, molluscs, odonata and fishes. Of the 23,291 species assessed by 2013, 226 freshwater species are thought to be Extinct, 16 are Extinct in the Wild and 5,320 are threatened with extinction (Critically Endangered, Endangered or Vulnerable). Supporting the Red List assessments are over 20,000 distribution maps for freshwater species that provide an ever-improving spatial understanding of freshwater species diversity. IUCN freshwater species distributions are mapped to HydroBASINS sub-catchments such that the scale of spatial information is matched to that required to underpin practical conservation decisions and management. Initiatives to identify freshwater Key Biodiversity Areas are now made possible by improvements in spatial data on diversity, threat and endemism. We review the most comprehensive collection of distribution information on global freshwater species. These advances in knowledge will not only inform but raise awareness and garner support for safeguarding freshwater biodiversity.