Background - The update of the global burden of disease attributable to the environment is presented. The study focuses on modifiable risks to show the potential health impact from environmental interventions. Methods - Systematic literature reviews on 133 diseases and injuries were performed. Comparative risk assessments were complemented by more limited epidemiological estimates, expert opinion and information on disease transmission pathways. Population attributable fractions were used to calculate global deaths and global disease burden from environmental risks. Results - Twenty-three percent (95% CI: 13–34%) of global deaths and 22% (95% CI: 13–32%) of global disability adjusted life years (DALYs) were attributable to environmental risks in 2012. Sixty-eight percent of deaths and 56% of DALYs could be estimated with comparative risk assessment methods. The global disease burden attributable to the environment is now dominated by noncommunicable diseases. Susceptible ages are children under five and adults between 50 and 75 years. Country level data are presented. Conclusions - Nearly a quarter of global disease burden could be prevented by reducing environmental risks. This analysis confirms that eliminating hazards and reducing environmental risks will greatly benefit our health, will contribute to attaining the recently agreed Sustainable Development Goals and will systematically require intersectoral collaboration to be successful.