Aim: The aim of this case report is to discuss the features of molar incisor hypomineralization (MIH), a developmental enamel defect, which was noted in a set of monozygotic twin males. Summary: MIH is the hypomineralization of one to four first permanent molars (FPMs) frequently associated with affected incisors and varies in clinical severity. Early diagnosis and management of the condition is paramount and will avoid the premature loss of the molars. Key learning points: The clinical characteristics, as well as the short and long term management of this condition are discussed.