The prevalence rate of congenital microtia varies greatly across the globe, ranging from 0.83/10 000 to 17.4/10 000. According to the Report on the Prevention and Treatment of Birth Defects in China (2012) issued by the former Ministry of Health, congenital microtia ranks 10th in the incidence rate, and there is a rising trend year by year, which impose huge physical obstacles, as well as psychological and economic burdens on patients. In recent years, as the problem of air pollution becomes increasingly serious, more and more studies focus on the impact of air pollutants on population health, and some studies link air pollution with ear deformities. After searching, reading, sorting, and analyzing a large number of literature, we reviewed studies on the prevalence of congenital microtia, the relationship between air pollution and congenital microtia, and related etiological mechanism. We confirmed their relationship, and discussed the etiological clues, aiming to provide insights for the etiological study on congenital microtia and the formulation of preventive measures.