BACKGROUND: In recent years, vitamin D in the occurrence of lung diseases has gradually become a hot topic. Although the role of vitamin D in normal lung development has been confirmed, the correlation between vitamin D level and neonatal respiratory distress syndrome (NRDS) is not clear. OBJECTIVE: To evaluate the association between vitamin D level and NRDS. METHODS: We performed a comprehensive search of the following electronic databases: PubMed, Embase, Cochrane Library, Web of Science, and China National Knowledge Infrastructure (CNKI). Literature screening and quality assessment were performed according to inclusion and exclusion criteria. The Newcastle-Ottawa Scale was used to assess the methodological components of each study, and Stata 15.1 software to perform the Meta-analysis. RESULTS: A total of nine case-control studies were included, with 653 infants with NRDS and 501 infants without NRDS. The Meta-analysis showed no heterogeneity across all studies(I(2)=0.0%, P=0.583). The fixed-effect model showed that 25 hydroxy vitamin D level of children in the NRDS group was significantly lower than that of the non-NRDS group(SMD = -0.51, 95%CI: -0.63 to -0.39, p ≤ 0.05). CONCLUSION: This systematic review and meta-analysis study suggests that vitamin D deficiency is very likely to be a high-risk factor of NRDS, and reasonable vitamin D supplementation during pregnancy and after birth is of great significance.