An international perspective of the magnitude of anemia in indigenous peoples is currently lacking. The present systematic review was performed to characterize the global prevalence, severity, and etiology of anemia in indigenous peoples by conducting a systematic search of original research published in English from 1996 to February 2010 using PubMed, Medline, and Embase. A total of 50 studies, representing the following 13 countries, met the inclusion criteria: Australia, Brazil, Canada, Guatemala, India, Kenya, Malaysia, Mexico, New Zealand, Sri Lanka, Tanzania, the United States, and Venezuela. Results indicate major deficiencies in the coverage and quality of anemia monitoring data for indigenous populations worldwide. The burden of anemia is overwhelmingly higher among indigenous groups compared to the general population and represents a moderate (20-39.9%) to severe (≥40%) public health problem. For the most part, the etiology of anemia is preventable and includes inadequate diet, poor living conditions, and high infection rates (i.e., malaria and intestinal parasites). A concerted global effort is needed to reduce the worldwide burden of anemia in these marginalized populations.