Molar and incisor hypomineralization is a developmental defect that is systemic in origin that affects one or more than one permanent first molars, and is often associated with permanent incisors. It is usually characterized by well demarcated opacities and qualitative enamel defects caused by decreased inorganic enamel components, and reduced mineralization. It can cause esthetic, functional, psychological, and behavioral problems in children. Its reported prevalence varies widely, from 2.5% to 40.2%. Multiple aspects of dental treatment for it are challenging, such as behavior management, difficulty in achieving adequate local anesthesia, tooth hypersensitivity, and retention of restorations. This review discusses the most important considerations pertaining to its prevalence, severity, etiology, differential diagnosis, and some of the challenges and treatment modalities applicable in young patients. Data is collected from PubMed, Medline, and Embase databases.