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Ambient temperature is an important determinant of mortality and morbidity, making it necessary to assess temperature-related burden of disease (BD) for the planning of public health policies and adaptive responses. To systematically review existing epidemiological evidence on temperature-related BD, we searched three databases (PubMed, Web of Science, and Scopus) on 1 September 2018. We identified 97 studies from 56 counties for this review, of which 75 reported the fraction or number of health outcomes (include deaths and diseases) attributable to temperature, and 22 reported disability-adjusted life years (include years of life lost and years lost due to disability) related to temperature. Non-optimum temperatures (i.e., heat and cold) were responsible for >2.5% of mortality in all included high-income countries/regions, and >3.0% of mortality in all included middle-income countries. Cold was mostly reported to be the primary source of mortality burden from non-optimum temperatures, but the relative role of three different temperature exposures (i.e., heat, cold, and temperature variability) in affecting morbidity and mortality remains unclear so far. Under the warming climate scenario, almost all projections assuming no population adaptation suggested future increase in heat-related but decrease in cold-related BD. However, some studies emphasized the great uncertainty in future pattern of temperature-related BD, largely depending on the scenarios of climate, population adaptation, and demography. We also identified important discrepancies and limitations in current research methodologies employed to measure temperature exposures and model temperature-health relationship, and calculate the past and project future temperature-related BD. Overall, exposure to non-optimum ambient temperatures has become and will continue to be a considerable contributor to the global and national BD, but future research is still needed to develop a stronger methodological framework for assessing and comparing temperature-related BD across different settings.