Objective: The purpose of this literature review is to compile updated information on the diagnostic and therapeutic guidelines for molar incisor hypomineralization. Material and methods: We searched through PubMed, Embase, Ovid, Cochrane Central and Google Scholar and identified 759 studies; 64 evaluated in full text that included 14 clinical trials. Of the included studies, 10 discussed different treatment modalities for molars affected by molar incisor hypomineralization. Conclusion: This updated information recommends, on one hand, focusing the different therapeutic options based on the need for treatment index and, on the other, starting treatment as early as possible to eliminate the risk of caries and its consequences in these patients.