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<b>Background/Objectives:</b> This systematic review aims to investigate the potential association between vitamin D levels and the occurrence of developmental enamel defects (DDE) in children, including conditions like molar-incisor hypomineralization (MIH) and hypomineralized second primary molars (HSPMs). DDEs, which occur during tooth development, can result in significant aesthetic and functional issues, and their exact etiology remains unclear, with both genetic and environmental factors contributing. Among environmental factors, vitamin D deficiency has been proposed as a possible risk factor, given its role in enamel mineralization. <b>Methods:</b> A thorough literature search was conducted in PubMed, Scopus, and Embase. The search strategy included terms such as "vitamin D", "vitamin D deficiency", "developmental defects of enamel", "enamel hypoplasia", "molar-incisor hypomineralization", and "hypomineralized second primary molars". Studies were included if they were original human observational research (cohort, case-control, or cross-sectional) conducted in children under 18 years of age or involving maternal-child cohorts. Ten studies were included in the analysis, with a total of 15,891 participants. The primary data extracted from the selected studies included the following: study design, participants' age, sample size, vitamin D status in relation to developmental defects of enamel, and statistical significance <b>Results:</b> The findings were mixed, with only a few studies suggesting a significant association between low vitamin D levels and the presence of DDEs. Specifically, one study found a link between insufficient maternal vitamin D levels during pregnancy and an increased number of teeth affected by MIH in children. However, the majority of the studies did not report a significant association. <b>Conclusions:</b> This review concludes that while there is some evidence to suggest a possible relationship between vitamin D and DDEs, more research is needed to confirm these findings and better understand the underlying mechanisms.