While very rare, catastrophic injuries in youth sports have a major impact on athletes and their families when they do occur. This article reviews and summarises the sparse research on direct catastrophic injuries in youth sports, a direct catastrophic sports injury being defined as a sport injury that resulted from participation in the skills of the sport, and resulted in a fatality or in a non-fatal brain or spinal cord injury, or skull or spinal fracture. While an electronic database search was completed to assemble the articles reviewed here, much of the data come from the National Center for Catastrophic Injury Research at the University of North Carolina, which has the most extensive and complete data set on this issue. This article reviews and summarises what is known about the rate of occurrence of these injuries in various youth sports, the risk factors for these injuries, injury mechanisms and what can be done to prevent them in various youth sports.