Purpose Osteogenesis imperfecta (OI) is characterized by increased bone fragility and susceptibility for fractures. A few studies described and compared treatment modalities for femur fractures in children with OI. However, no cohort studies on adults with OI have been published. This study on adult OI patients aims to give insight into the incidence of femur fractures and non-unions and its best treatment options to avert non-union. Methods In this retrospective, descriptive study of the OI expert clinic in The Netherlands, all medical charts of patients 16 years or older were analyzed for femur fracture incidence, non-union rate and treatment modality. Results Of 216 OI patients, 34 patients suffered a femur fracture with 12 patients having more than 1 femur fracture. For all types of femur fractures, the incidence was 651 fractures per 100,000 person-years annually. In 49 total fractures, 10 fractures resulted in a non-union, mostly shaft fractures of type 4 OI patients. Surgically treated shaft fractures had the best outcomes for non-union. Conclusions OI adults were prone to developing femur fractures and non-unions. Especially type 4 OI adults, with conservatively treated shaft fractures, were at high risk for non-unions.