In this paper, the current knowledge about Molar Incisor Hypomineralization (MIH) is presented. MIH is defined as hypomineralization of systemic origin of one to four permanent first molars frequently associated with affected incisors and these molars are related to major clinical problems in severe cases. At the moment, only limited data are available to describe the magnitude of the phenomenon. The prevalence of MIH in the different studies ranges from 3.6-25% and seems to differ in certain regions and birth cohorts. Several aetiological factors (for example, frequent childhood diseases) are mentioned as the cause of the defect. Children at risk should be monitored very carefully during the period of eruption of their first permanent molars. Treatment planning should consider the long-term prognosis of these teeth.