A molar-incisor malformation (MIM) is a newly discovered type of dental anomaly of the permanent first molars, deciduous second molars, and permanent maxillary central incisors. MIM anomalies of the permanent first molars and deciduous second molars may include normal crowns with a constricted cervical region and thin, narrow, and short roots, whereas the affected maxillary central incisors may exhibit a hypoplastic enamel notch near the cervical third of the clinical crown. Although the etiology of MIM remains to be determined, it is thought to be attributable to an epigenetic factor linked to brain- and central nervous system–related systemic diseases at around age 1 to 2 years. MIM teeth are associated with clinical problems such as impaction, early exfoliation, space loss, spontaneous pain, periapical abscess, and poor incisor esthetics. Children with MIM teeth should be observed closely with respect to their medical history, and dentists should formulate a wider-ranging treatment plan.