Osteogenesis imperfecta (OI) is a genetic disorder that affects all connective tissue. Clinical manifestations of OI include bone fragility, hyperlaxity of joints, hearing loss, abnormalities of stature and facial structure, blue sclerae, and dentinogenesis imperfecta (DI). OI is classified into four groups according to the severity and physical characteristics of the disease, although not all characteristics may be present in one individual. Currently, 20,000 to 50,000 individuals in the U.S. have been diagnosed with this disease. The aim of this article is to discuss medical and dental complications associated with OI and DI. A case presentation describes the clinical care of a patient from birth to age 12.