Molar incisor hypomineralization (MIH) is a developmental condition affecting the enamel, primarily targeting one to four permanent first molars, often with the involvement of permanent incisors. The condition is characterized by distinct white-yellow or yellow-brown opacities, with more severe cases exhibiting hypomineralized enamel that is prone to breakdown. Recent data highlights MIH as a widespread dental issue seen across the globe. Despite its prevalence, the exact etiology remains unclear due to the variety of potential contributing factors. Managing MIH is particularly challenging, requiring a holistic approach to address the broad spectrum of symptoms and the heightened sensitivity of the affected teeth. Given the unique challenges of MIH, it is essential to gather updated and thorough knowledge. This understanding is critical for exploring potential preventive measures and enhancing treatment outcomes. This review aimed to examine the underlying causes of MIH, assess various treatment strategies, and offer a thorough understanding of the condition based on the latest research.