This paper presents a review of dentinogenesis imperfecta occurring in patients with osteogenesis imperfecta. The systemic manifestations and the oral aspects of dentinogenesis imperfecta in osteogenesis imperfecta are discussed, and an illustrative case is described. Keywords: case report, dental anomalies, dentinogenesis, dentinogenesis imperfecta, human dentin, osteogenesis imperfecta