Osteogenesis imperfecta is thought to be characterized by good bone union despite bone insufficiency causing fracture and deformity. We report two female patients who underwent surgical treatment for pseudoarthrosis of the femur with mild osteogenesis imperfecta who had gone without treatment for a long period of time. They were 12 and 31 years old at the time of surgery. Each fractured their femur on one side, one at 4 and one at 2 years of age, but had not been treated due to lack of pain. As a result, they exhibited limping, shortening of the leg, and bulging of the proximal thigh just before surgery. Muscle release around the hip and bone resection followed by correction with Ender nailing in the region with pseudoarthrosis was performed. Good bone fusion was achieved in both cases. To obtain good bone fusion, fixation with Ender nailing should be considered for pseudoarthrosis of the femur complicated by osteogenesis imperfecta. Correction of the mechanical axis and discrepancy of lower extremities are important in addition to the correction of deformity.
