Osteogenesis imperfecta is a syndrome of interest to dentists because dentinogenesis imperfecta is frequently associated with not only bone disease but also tooth disease. Specifically, these patients usually have many dental problems such as 1) high incidence of tooth fracture, enamel chipping and cracking, 2) high caries activity, 3) inability or difficulty of endodontic treatment, 4) masticatory disturbance and 5) esthetic dissatisfaction. This paper presents a case report of a patient with osteogenesis imperfecta and dentinogenesis imperfecta who was provided prosthetic treatment, including endodontic treatment of the obliterated teeth.