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Osteogenesis imperfecta is a very rare heterogeneous genetic disorder associated with the development of connective tissue resulting in fragile bones and frequent fractures. More than 50% of patients affected with osteogenesis imperfecta have a hereditary developmental disorder known as dentinogenesis imperfecta. Dentinogenesis imperfecta is caused by irregularities in the formation, composition and organization of dentin matrix during tooth development. It is caused by mutations of the genes that encode basic proteins of the organic matrix, collagens and phosphoproteins. The purpose of this review is to describe the histopathologic and clinical features of teeth typical of dentinogenesis imperfecta type I, which occurs within osteogenesis imperfecta, with special emphasis on targeted dental treatment to achieve optimal rehabilitation of the masticatory system. [ABSTRACT FROM AUTHOR]