Many sports have been associated with a variety of neurological injuries affecting the central nervous system (CNS), with some injuries specific to that sport. A systematic review of sport-specific CNS injuries has not been attempted previously, and could assist in the understanding of morbidity and mortality associated with particular sporting activities, either professional or amateur. A systematic review of the literature was performed using PubMed (1965-2003) examining all known sports and a range of possible CNS injuries attributable to that sport. Numerous sporting activities (45) have associated CNS injuries as reported within the literature. The sports most commonly associated with CNS injuries are: football, boxing, hockey, use of a trampoline, and various winter activities. A number of sporting activities are associated with unique CNS injuries or injury-related diseases such as heat stroke in auto racing, vertebral artery dissection in the martial arts, and dementia pugilistica in boxing. Neurological injuries of the CNS due to sport comprise a wide collection of maladies that are important for the neurologist, neurosurgeon, orthopaedic surgeon, physiatrist, sports medicine doctor, athletic trainer and general physician to recognise.