Neural tube defects(NTDs)is a common neurological disease in the world, with an occurrence of about1/ 1000. The pathogenesis of this disease is complicated, which is determined by genetic factors and environmental factors.Till now, there is no effective treatment, however supplying folic acid can reduce the incidence of neonatal NTDs. Presentstudies focused mainly on zebrafish, Xenopus and mouse experiments, and found that the planar cell polarity andconvergent extension play an important role in neural tube closure. To better understand the function and molecularmechanism of PCP pathway in the process of neural tube closure, this review summarized the current progress in research ofthe disease and the relationship between the disease and three genes: SHROOM3, VANGL2 and WNT5A, in addition summarized the animal models of NTDs.