The findings are based on a clinical investigation conducted on forty-nine patients suffering from osteogenesis imperfecta (OI), as well as on a questionnaire study in which 117 osteogenesis imperfecta-affected persons or their parents were involved. The survey established pathological tooth discolorations as well as tooth abrasions. Dentinogenesis imperfecta (DI) was more frequently found in primary teeth than in permanent teeth. There were no gender-specific differences. Radiological abnormalities were found in both, abraded and/or discolored teeth, as well as in clinically normal appearing teeth. In most cases there were club-shaped extensions of the pulp chambers and obliterations of the root canals. The probability that dentinogenesis imperfecta occurs as an accompanying symptom of osteogenesis imperfecta was not dependent on the degree of skeletal severity. The self-assignment according to A and B forms of osteogenesis imperfecta types I and IV in accordance with the presence/absence of dental symptoms was contradictory, since the literature was based on varying classifications.