The Arctic has been a subject to various socio-cultural changes; indigenous people living in the region have experienced injustice and oppression in different forms. Furthermore, there are currently various new social, political and environmental challenges. It has been assumed that the continuous socio-cultural transition has an influence on indigenous people's wellbeing. We conducted a systematic literature review with regard to epidemiological mental health research on Arctic indigenous children and adolescents. The aim was to describe the nature and scope of research conducted and to explore for possible regional and ethnic differences in mental health. It was found that current epidemiological knowledge is based mainly on cross-sectional studies from selected regions and limited to substance use and suicidal behaviour. Youth suicide rates are alarmingly high in many parts of the Arctic, particularly in Greenland and Alaska. Differences between indigenous and non-indigenous groups are also most evident and uniform across suicide studies, with rates being systematically higher among indigenous youth. Substance use is common throughout the Arctic, however, regional and ethnic differences in usage vary considerably. Other psychosocial problems remain largely unexplored. In addition, very little is known about the causes of mental health problems in general and the impact of rapid socio-cultural changes in particular. There are several methodological limitations in the studies included here, many related to the validity of research instruments in different cultural contexts. There is a need for longitudinal comparative studies from the entire Arctic with culturally relevant instruments addressing mental health in early childhood as well.