MIH was defined by Weerheijm (2001) as "hypomineralisation of systemic origin of 1-4 permanent first molars, frequently associated with affected incisors". The prevalence of MIH varies between 2.8% and 25%, dependent upon the study. At their sixth congress in 2003, The European Association of pediatric dentistry defined criteria for diagnosis of the phenomena. It included the presence of demarcated opacity, posteruptive enamel breakdown, atypical restoration, extracted molar due to MIH and unerupted teeth. According to the teeth involved and to the time of the crown formation, researches focused on environmental and systemic conditions as possible reasons for MIH.The etiologies were divided into five groups: Exposure to environmental contaminants, pre/peri and neonatal problems, exposure to fluoride, common childhood illnesses and medically compromised children. The clinical implications include highly sensitive teeth, difficulty to achieve adequate anesthesia, behavioral problems and anxiety, rapid progression of caries and the esthetic implications. A six step approach to management was described suggested: risk identification, early diagnosis, remineralization and desensitization, prevention of caries and posteruption breakdown, restorations and extractions and finally maintenance. Restoring an affected molar can vary from adhesive intra coronal restorations (resin composite is the material of choice) to extra coronal restorations (e.g. preformed metal crown). Esthetic solutions to affected incisors may include microabrasion (that shows little improvement) and resin composite or porcelain veneer. The key for a successful treatment is early diagnosis, intense follow up and usage of remineralizating agents as soon as the teeth erupt. There is still need for further research to clarify the etiological factors and improve the durability of restoration in affected teeth.