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Background: Diabetes mellitus (DM) is a common chronic disorder in children and is caused by absolute or relative insulin deficiency, with or without insulin resistance. There are several different forms of childhood DM. Children can suffer from neonatal diabetes mellitus (NDM), type 1 diabetes (T1DM), type 2 diabetes (T2DM), Maturity Onset Diabetes of the Young (MODY), autoimmune monogenic, mitochondrial, syndromic and as yet unclassified forms of DM. The Middle East has one of the highest incidences of several types of DM in children; however, it is unclear whether pediatric diabetes is an active area of research in the Middle East and if ongoing, which research areas are of priority for DM in children. Objectives: To review the literature on childhood DM related to research in the Middle East, summarize results, identify opportunities for research and make observations and recommendations for collaborative studies in pediatric DM. Methods: We conducted a thorough and systematic literature review by adhering to a list recommended by PRISMA. We retrieved original papers written in English that focus on childhood DM research, using electronic bibliographic databases containing publications from the year 2000 until October 2018. For our final assessment, we retrieved 429 full-text articles and selected 95 articles, based on our inclusion and exclusion criteria. Results: Our literature review suggests that childhood DM research undertaken in the Middle East has focused mainly on reporting retrospective review of case notes, a few prospective case studies, systemic reviews, questionnaire-based studies, and case reports. These reported studies have focused mostly on the incidence/prevalence of different types of DM in childhood. No studies report on the establishment of National Childhood Diabetes Registries. There is a lack of consolidated studies focusing on national epidemiology data of different types of childhood DM (such as NDM, T1DM, T2DM, MODY, and syndromic forms) and no studies reporting on clinical trials in children with DM. Conclusions: Investing in and funding basic and translational childhood diabetes research and encouraging collaborative studies, will bring enormous benefits financially, economically, and socially for the whole of the Middle East region., (Copyright © 2019 Saraswathi, Al-Khawaga, Elkum and Hussain.)