Wheelchair racing is one of the most popular sporting activities of individuals with spinal cord injury. Athletes with this impairment have unique changes in metabolic, cardiorespiratory, neuromuscular and thermoregulatory systems, which reduce their overall physiological capacity compared with able-bodied individuals or individuals with other types of impairments. This review on spinal cord injury: presents the International Stoke Mandeville Games Federation classification of wheelchair athletes; describes methods commonly used to characterise anaerobic and aerobic fitness; presents the findings of physiological studies that have evaluated wheelchair racing performance; identifies the risks associated with temperature regulation when competing in wheelchair races; and discusses special conditions that can influence wheelchair racing performance. Currently there is limited research that has examined the relationship between sprint or distance wheelchair racing performance and the anaerobic and aerobic components of physical fitness. Although the descriptive evidence indicates that the profiles of these athletes reflect their training and participation in these specific events, the association between their physiological profiles and real or simulated racing performance is unclear. The generally accepted concept that high values of aerobic and anaerobic power are strongly correlated with endurance and sprint racing performance, respectively, are not necessarily true in this population. Athletes with spinal cord injury have an impaired thermoregulatory capacity, because the compromised autonomic and somatic nervous system functions disrupt control of skin blood flow and sweating below the level of the lesion. As a result, they may be more susceptible to hyperthermia during distance wheelchair racing performance. Wheelchair athletes should follow recommendations advocated for able-bodied individuals to minimise their risks of heat stress during competition. Many athletes with quadriplegia voluntarily induce autonomic dysreflexia (commonly known as boosting) during distance racing events to improve performance. Experimental evidence indicates that boosting can improve performance time by 10% in elite wheelchair marathon racers during simulated racing, as a result of increased oxygen utilisation in the boosted state. However, since boosting can be dangerous to health, the International Paralympic Committee has banned athletes from voluntarily inducing it during competition. The use of anti-gravity suits to increase lower-body positive pressure can increase the peak oxygen uptake, cardiac output and stroke volume. However, the use of abdominal binders does not influence these physiological responses. An effect of either of these techniques on wheelchair racing performance has not been demonstrated.