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Background: Diabetes is referred to a group of diseases characterized by high glucose levels in blood. It is caused by a deficiency in the production or function of insulin or both, which can occur because of different reasons, resulting in protein and lipid metabolic disorders. The aim of this study was to systematically review the prevalence and incidence of type 1 diabetes in the world. Methods: A systematic search of resources was conducted to investigate the prevalence and incidence of type 1 diabetes in the world. The databases of Medline (via PubMed and Ovid), ProQuest, Scopus, and Web of Science from January 1980 to September 2019 were searched to locate English articles. The located articles were screened in multiple levels of title, abstract, and full-text and final studies that met the inclusion criteria were retrieved and included in the study. Results: From 1202 located articles, 193 studies were included in this systematic review. The results of meta-analysis showed that the incidence of type 1 diabetes was 15 per 100,000 people and the prevalence was 9.5% (95% CI: 0.07 to 0.12) in the world, which was statistically significant. Conclusion: According to the results, the incidence and prevalence of type 1 diabetes are increasing in the world. As a result, insulin will be difficult to access and afford, especially in underdeveloped and developing countries. [ABSTRACT FROM AUTHOR]