Abstract: Zinc is an essential trace element. Zinc insufficiency or deficiency is a global nutritional problem. Recently many studies have shown that maternal zinc insufficiency or deficiency during gestation may be associated with several major birth defects. In this review, we summarized the results in the literature regarding the association between maternal zinc level and major birth defects from both animal experiments and human epidemiological studies. It is concluded that maternal zinc insufficiency or deficiency may be a risk factor for congenital heart diseases, orofacial clefts and neural tube defects. The possible teratogenic mechanisms underlying the association include apoptosis and cell cycle disturbances, oxidative stress as well as cell signaling alternation.