Cancer mortality among indigenous peoples is increasing, but these populations commonly under use cancer-screening services. This systematic review explores knowledge, attitudes, and behaviours towards cancer screening among indigenous peoples worldwide. Searches of major bibliographic databases identified primary studies published in English up to March, 2014; of 33 eligible studies, three were cohort studies, 27 cross-sectional, and three case-control. Knowledge of and participation in screening was greater for breast cancer than for other cancers. Indigenous peoples tended to have less knowledge, less favourable attitudes, and a higher propensity to refuse screening than non-indigenous populations. The most common factors affecting knowledge, attitudes, and behaviours towards cancer screening included access to screening, knowledge about cancer and screening, educational attainment, perceived necessity of screening, and age. Greater understanding of knowledge, attitudes, and behaviours towards cancer screening in diverse indigenous cultures is needed so that culturally appropriate cancer prevention programmes can be provided.