Key Clinical MessageThe etiology of neural tube defects is multifactorial, with a wide interplay of genetic and environmental factors. However, periconceptional folic acid should be supplemented in antenatal care.AbstractWe described a case of neural tube defects (NTDs), specifically occipital encephalomeningocele, in a child born to a mother who received folic acid supplementation. A wide interplay of genetic and environmental factors exists in its causation. Although folic acid confers advantage, the relationship with the causation of NTDs still remains unclear.