Osteofibrous dysplasia occurs most frequently in the tibia and may result in deformity and pathological fracture. We report one such case in a 6-year-old girl who underwent minimally invasive plate osteosynthesis using a locking compression plate. The varus deformity of the tibia was manually corrected, and the plate was inserted without extensive surgical exposure. At 20-week follow-up, the fracture had healed completely, and she had returned to normal daily living. At 3-year follow-up, radiographs showed no progression of the varus deformity. Minimally invasive plate osteosynthesis is useful for treatment of pathological fractures caused by osteofibrous dysplasia. It preserves blood flow at the fracture site, leading to good bone healing and stability to prevent further deformity and an early return to daily activity.