The etiology of neural tube defects, a category encompassing spina bifida, anencephaly and encephalocele, remains highly controversial and unclear, However, there is overwhelming evidence supporting a multifactorial etiology for this group of defects. Recent studies have shown that folic acid supplements taken periconceptionally can reduce a woman's risk of having a child with a neural tube defect. Qenetic screening could identify women who will require folic acid supplements periconceptionally to reduce their risk of having a child with a neural tube defects. Recently, we encountered two cases of recurrent anencephaly that had occurted in a same pregnant woman in our hospital. We report these two cases with brief review of the literatures.