In recent years, bisphosphonates, primarily intravenous (iv) pamidronate, have become very widely used in children with severe osteogenesis imperfecta (OI). This has occurred without the well-controlled clinical trials usually required before a medication becomes ‘standard care’. Without placebo-controlled trials it is difficult to define precisely the risks and the benefits, but in children with very severe OI and numerous fractures the risk/benefit ratio is so clearly favorable that widespread use of pamidronate in this situation appears appropriate.