Background: In human birth defects, NTDs account for 0.5-2 per 1000 pregnancies worldwide. It occurs due to abnormalities in neural tube formation. Zinc required for the absorption and synthesis of biologically active folate moiety. Its deficiency creates a lack of folate in metabolic activity. Lead competes with zinc and slowly decreases zinc stores in the body. It causes morphological changes and gives an incomplete blood-brain barrier to the developing nervous system. Objective: The objective of the study was to find out the associations between maternal serum lead concentration and fetal NTDs. Materials and Methods: It was a case-control study conducted in Feto- Maternal Medicine Unit, Department of Obstetrics and Gynecology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from January 2016 to June 2016. Ethical clearance was taken. A total of 60 pregnant women were selected as the study population. 30 with USG detected fetal NTDs case and 30 with normal fetus. In both groups folic acid level was normal. Maternal serum was taken for measuring lead by Graphite furnace automatic absorption spectrophotometer. Data were analyzed by SPPS version 17. Results: The raised value of serum lead was found in 6.67% of patient with fetal NTD. Relationship between NTD and raised value of lead were found significant. Conclusion: From this study, we concluded, raised serum lead level is one of the associated factors for fetal neural tube defects.