Molar incisor hypomineralization (MIH) is a congenital disease which is increasing in prevalence. It affects permanent first molars and, often to a lesser degree, permanent incisors with variable severity. The etiology is unknown, but various risk factors have been put forward. Differential diagnosis is mandatory not to confound MIH with other diseases such as amelogenesis imperfecta or fluorosis. Treatment consists in a minimally invasive approach by reinforcing and protecting the existing dental structure. In more severe cases, restorative treatment or extraction may be indicated.