Background Indigenous children suffer a disproportionally high burden of unintentional injuries. A more detailed understanding of the underlying causes, risk factors and gaps in research is required to inform prevention efforts and direct future research. The aim of this review was to systematically assess the evidence regarding differences in rates of unintentional injuries between indigenous and non-indigenous children and to identify leading causes and underlying risk factors contributing to these differences. Method We systematically searched the literature including 10 electronic databases, institutional websites and reference lists of relevant studies. Due to the substantial heterogeneity between studies, results were summarised in a narrative synthesis and no metaanalysis was carried out. Results A total of 39 studies were included in this review. Most studies were descriptive and only five adjusted for potential confounding in the analysis. Indigenous to non-indigenous rate ratios for morbidity and mortality for unintentional injury ranged from 1.2 to 2.3 and 1.8 to 8.2, respectively. The difference varied greatly by cause of injury and between studies, ranging from a reduced risk of hospitalisation due to fall injuries to a 17-fold increased risk of mortality due to pedestrian injuries. Burns, poisoning and transport injuries were the major contributors to the increased injury burden in indigenous children. The studies offered only limited insight into the underlying causes of these differences, but socioeconomic status and parents’ educational attainment were contributing factors. Conclusions Indigenous children experience a significantly higher burden of morbidity and mortality from unintentional injuries across different indigenous communities worldwide. Most of these injuries are highly preventable, presenting substantial potential to improve indigenous child health. However, there is limited evidence to illuminate the underlying risk factors for unintentional injuries in indigenous children, and this is a priority for further research.