摘要: Development of teeth is under strict genetic control, which ensures the formation and renewal a certain number with specific shapes positions in reproducible timetable. Gene mutations can disturb normal dental development affect tooth number, shape, eruption or hard tissues, enamel, dentin cementum. These anomalies are often observed as isolated, that is, only dentition affected, especially failure to develop all teeth, agenesis hypodontia extremely common. Different also numerous rare developmental syndromes because genes networks regulate morphogenesis differentiation active other organs tissues. Tooth associated ectodermal defects orofacial clefts. Many examples indicate most sensitive reduced activity pathway related complexity self-organising features development. Key Concepts: Teeth specialised organs. Development regulated by cell interactions same signalling pathways requires modulation antagonist factors. Tooth (failure teeth) one common anomalies. Most cases supernumerary caused rather than complete inactivation gene activity. Dental diagnostic indications heritable syndrome systemic disease. Dental tissues do not regenerate after an injury their involve unique mechanisms. Studies into congenital enamel revealing functions for novel proteins involved cellular regulation ameloblasts cells. Keywords: tooth agenesis; hypodontia; oligodontia; ectodermal dysplasia; cleft lip; cleft palate; enamel; dentin; tooth