Osteogenesis imperfecta is a group of systemic connective tissue diseases characterized by increase in bone fragility and collagen metabolic disorder. The condition is mainly caused by mutations in the genes encoding type Ⅰ collagen. The clinical features of the diseases include bone fragility, progressive bone and joint deformity, blue sclera, dentinogenesis imperfecta and hearing loss. Currently, the most promising drug for treatment of the disease is bisphosphonate. This article reviews the following issues: optimal dose and duration of treatment, best route for administration, best drug choice, and the optimal match of drugs with the osteogenesis imperfecta type and age of patients.