The management of molar incisor hypomineralization (MIH) is considered challenging for patients, parents and the dentist; it has a systemic origin, it can occur in one or more permanent molars, incisors can be affected simultaneously. These enamel defects occur due to several risk factors during the pre-, peri- and postnatal period. Objective: To analyze the literature on molar-incisor hypomineralization and its relationship with prenatal risk factors, diagnosis, enamel remineralization techniques and treatment. Methodology: Using the keywords “molar incisor hypomineralization”, “etiology”, “diagnosis”, “oral manifestations”, “enamel remineralization” and “clinical management”, the Medline/PubMed and Science Direct databases were searched, with emphasis on the last 5 years. It was evaluated with the PRISMA and AMSTAR-2 guidelines. Results: Postnatal factors are based on childhood diseases and antibiotic intake in the developmental stages of the teeth. In the prenatal period, to diseases during pregnancy. Perinatal factors are related to low birth weight. MIH is evaluated depending on the severity based on defects, color, location and post-eruptive collapse and the extent of the affected surface. Techniques to remineralize the enamel are only efficient to avoid caries progression. Microabrasion is the most commonly used treatment technique. Conclusion: Most of the risk factors are attributed to the postnatal period during the first three years of life and HMD increases the prevalence of MIH.