Osteogenesis imperfecta during pregnancy presents increased risk in mother and fetus. In addition lo the known skeletal changes', other recently recognized metabolic abnormalities also may lead lo maternal an leis during labor and delivery. A discussion of risk factors and their management is presented. The importance of considering the possibility of fetal osteogenesis imperfecta is stressed and cesarean is presented at the method of choice for delivery. Important genetic factors including the lack of correlation between the severity of involvement of parent and offspring and the use of pyrophosphate analyses are discussed. An illustrative case is presented.