Osteogenesis Imperfecta (OI) is an inherited skeletal dysplasia characterized from bone fragility and skeletal deformities. OI presents with heterogenous features and variability in severity. Multiple systems are affected, since the disease involves the synthesis of collagen type I. Diagnosis is mainly based on the incidence of fractures. Severe osteoporosis, repeated fractures and fragility affect the skeleton and severe deformities are the result. Spine deformities are common. Muscle function and mobility is affected. Medical treatments including biphosphonates, denosumab, TGFb inhibitors are reported to increase bone mass, but fractures remain the main consideration of OI. Surgical treatment with the use of intramedullary expanding rods increases bone strength, correct deformities and provide stability for the mobilization of the affected patients. These procedures have a high rate of complications but have significantly improved the quality of life of the affected children.