Tooth agenesis, belonging to the abnormal tooth development, is a common disease in clinic. The disease not only affects the person's chewing function, but also influences the pronunciation, appearance and mental health. In the past, genetic linkage and molecular biology research have made clear of part of the genetic mutations' sites of the syndromic and non-syndromic tooth agenesis. Although the mechanism was not clear yet, but the important mutations are now known to be involved in many factors. Thesyndromic and non-syndromic tooth agenesis related gene are reviewed in this paper.