Objective: Osteogenesis imperfecta (OI) is a rare genetic disease characterized by osteoporosis and fragility of the bones. These patients often require anesthesia for orthopaedic surgery because of recurrent bone fractures. Our primary goal in this study was to evaluate the patients operated for OI to determine the factors that are associated with perioperative and postoperative morbidity. Methods: The patient files were retrospectively evaluated between 2008 and 2018. Demographic data, number and type of operations, the duration of the last operation, type of anesthesia, perioperative position, perioperative and postoperative fracture formation, and amount of bleeding were recorded. Additionally, the need for intensive care, if any, length of intensive care unit stays, and length of hospital stay were determined, and the effect of these factors on morbidity and mortality was investigated. Results: In this study, 44 patients with OI, who had undergone 105 operations, were included with a mean age of 11.07±7.70 years. We demonstrated that the presence of scoliosis (p=0.001), body mass index measurements (p=0.008), and higher number of operations (p=0.014) were significantly associated with morbidity. However, when we made the regression model, we reported that only the presence of scoliosis, appeared to be a significant model (p=0.002; odds ratio: 9.082). Scoliosis increased the risk of morbidity 9.082-fold, which was an independent risk factor. Conclusion: In our study, we demonstrated that the scoliosis had an effect that increases the risk of morbidity 9-fold, and that scoliosis is an independent risk factor. Considering these data, we suggest that all OI patients undergoing orthopaedic surgery should be radiologically screened for the presence of scoliosis before the operation.