Although the cancer burden in indigenous children has been reported in some countries, up to now, no international comparison has been made. We therefore aimed to assess the available evidence of the burden of childhood cancer in indigenous populations. We did a systematic review of reports on cancer incidence, mortality, and survival in indigenous children worldwide. Our findings highlight the paucity of accessible information and advocate the pressing need for data by indigenous status in countries where population-based cancer registries are established. The true extent of disparities between the burden in the indigenous community needs to be measured so that targeted programmes for cancer control can be planned and implemented.