Benign fibrous histiocytoma ( BFH ) mainly occurs in the soft tissues, and BFH of the bone is rare. The literatures at home and abroad are mostly case reports. In this paper 1 case of 13-year-old girl with pathological fracture of the femur as the ifrst symptom was reported, who was treated with complete curettage, bone allograft and locking plate ifxation. The lesion was conifrmed to be BFH of the femur by biopsy. She was followed up for 1 year after the operation. The fracture ends and bone grafting were well healed, and the movement of the affected leg was normal, with no evidence of recurrence. Because BFH of the bone was extremely rare, and was easy to be confused with osteofibrous dysplasia, giant cell tumors of bone and other benign or borderline lesions, its clinical features, imaging characteristics, pathological characteristics, differential diagnosis, diagnosis, and treatment were described and summarized in this paper.