Amputation of a limb may have a negative impact on the psychological and physical well-being, mobility and social life of individuals with limb amputations. Participation in sports and/or regular physical activity has a positive effect on the above mentioned areas in able-bodied individuals. Data concerning participation in sports or regular physical activity together with its benefits and risks for individuals with limb amputations are scarce. No systematic review exists that addresses a wide range of outcomes such as biomechanics, cardiopulmonary function, psychology, sport participation and sport injuries. Therefore, the aim of this article is to systematically review the literature about individuals with limb amputations and sport participation. MEDLINE (PubMed), EMBASE, CINAHL® and SportDiscus® were searched without time or language restrictions using free text words and MeSH terms. The last search date was 31 March 2010. Books, internet sites and references of included papers were checked for papers relevant to the topic under review. Papers were included if the research topic concerned sports and a minimum of ten individuals with limb amputations were part of the study population. Papers were excluded if they included individuals with amputations of body parts other than upper or lower limbs or more distal than the wrist or ankle, or if they consisted of case reports, narrative reviews, books, notes or letters to the editor. Title, abstract and full-text assessments were performed by two independent observers following a list of preset criteria. Of the 3689 papers originally identified, 47 were included in the review. Most of the included studies were older than 10 years and had cross-sectional designs. Study participants were generally younger and often had more traumatic amputations than the general population of individuals with limb amputations. Heterogeneity in population characteristics, intervention types and main outcomes made data pooling impossible. In general, sports were associated with a beneficial effect on the cardiopulmonary system, psychological well-being, social reintegration and physical functioning. Younger individuals with unilateral transtibial amputations achieve better athletic performance and encounter fewer problems when participating in sports compared with older individuals with bilateral transfemoral amputations. Regardless of their amputation level, individuals with limb amputations participate in a wide range of recreational activities. The majority of them were not aware of the sport facilities in their area and were not informed about available recreational activities. Sport prosthetic devices were used mostly by competitive athletes. For football, the injury rate and pattern of the players with an amputation were similar to those of able-bodied players. Individuals with limb amputations appear to benefit both physically and psychologically from participation in sports and/or regular physical activity. Therefore, sports should be included in rehabilitation programmes, and individuals with limb amputations should be encouraged to pursue a physically active life following hospital discharge.