Molar incisor hypomineralization (MIH) describes the clinical picture of hypomineralization of systemic origin affecting one or more first permanent molars (FPMs) that are associated frequently with affected incisors. Etiological associations with systemic conditions or environmental insults during the child's first 3 years have been implicated. The complex care involved in treating affected children must address their behavior and anxiety, aiming to provide a durable restoration under pain-free conditions. The challenges include adequate anaesthesia, suitable cavity design, and choice of restorative materials. Restorations in hypomineralized molars appear to fail frequently; there is little evidence-based literature to facilitate clinical decisions on cavity design and material choice. A 6-step approach to management is described: (1) risk identification; (2) early diagnosis; (3) remineralization and desensitization; (4) prevention of caries and posteruption breakdown; (5) restorations and extractions; and (6) maintenance. The high prevalence of MIH indicates the need for research to clarify etiological factors and improve the durability of restorations in affected teeth. The purpose of this paper was to describe the diagnosis, prevalence, putative etiological factors, and features of hypomineralized enamel in molar incisor hypomineralization and to present a sequential approach to management.