Molar incisor hypomineralisation (MIH) is a congenital enamel defect with multifactorial etiology characterized by qualitative enamel hypomineralisation that affects one or more first permanent molars and it also often includes permanent incisors. While the exact cause of it still isn’t known it is considered that the link between MIH and multiple genetic, epigenetic and systemic factors exists. The research didn’t specify which medical problems are included under the term ‘maternal illnesses‘ but it was established that they indeed are connected with the development of MIH during the prenatal period. Factors connected with MIH development during the perinatal period are: hypoxia, low birth weight, premature birth, cesarean section and birth complications. The use of antibiotics, measles, urinary tract infection, otitis, fever, gastric disorders, asthma, pneumonia, kidney diseases are also associated with MIH during the postnatal period. The Würzburg concept currently represents the most accurate guidelines for management of MIH. It consists of MIH-TNI classification and treatment plan. MIH-TNI divides MIH into 4 different stages while taking into account post eruptive enamel breakdown and hypersensitivity. Contemporary treatment plan options include: prophylaxis (fluoridation, TCP, CCP-ACP, fluoride varnish), non-invasive therapy (fissure sealing, low viscosity SIC, bleaching, microabrasion, resin infiltration, etch-bleach-seal technique), temporary short-term therapy (SIC, SIC plus Orthoband, SDF plus SIC, SDF), temporary long-term therapy (SSC crown, zirconia crown), permanent therapy (direct composite restoration, indirect restoration) and extraction. Options for management of hypersensitivity and remineralisation can only be chosen based on clinicians personal experience because their effectiveness has yet to be confirmed by additional studies. Also, additional research is needed in case of management options for incisors.