We present the anaesthetic and surgical management, and post-operative course of a patient with osteogenesis imperfecta which exemplifies the problems associated with this condition. The observed petechial haemorrhagic rash is not characteristic of the bleeding tendency in osteogenesis imperfecta nor characteristic of acute disseminated intravascular coagulation. Despite potential life-threatening complications the patient made a good recovery and was discharged 3 weeks after surgery.