报告了９例长骨骨化性纤维瘤，男３例，女６例；就诊年龄最小３岁，最大３８岁，平均１７．２２岁；胫骨７例，股骨１例，肱骨１例。临床主要症状表现为局部肿块和疼痛，Ｘ线表现为多囊性溶骨性病变，皮质膨胀变薄，无骨膜反在。本组９例中经临床及Ｘ线正确诊断者仅１例．结合文献报道的病例分析，发现该病虽有一定特征，但临床误诊率甚高，最易误诊为骨纤维异常增殖症。其误诊原因主要因为该病少见，且与骨纤维异常增殖症极相似；也与该病组织结构表现多样呈渐进发展的特性有关。病理检查是确诊的主要手段，有病理骨折者，要尽早活检，取瘤体中心部织，避免骨折修复反应对诊断的影响。复发病例的病理诊断，要以第１次活检材料为准，排除手术后反应对诊断的干扰。与骨纤维异常增殖症难以鉴别时，应多点取材，了解肿瘤全貌，以其主要在现定性。 Nine cases of ossifying fibroids of the long bone were reported, including 3 males and 6 females. The patients were the youngest 3 years old and 38 years old with an average of 17.22 years old. There were 7 cases of tibia, 1 of femur, and 1 of tibia. The main clinical symptoms were local mass and pain, X-ray showed polycystic osteolytic lesions, cortical swelling became thin, and no periosteum was present. Of the 9 cases in this group, only 1 was diagnosed by clinical and X-ray. Combined with the case analysis reported in the literature, it was found that although the disease has certain characteristics, the rate of clinical misdiagnosis is very high, and it is most likely misdiagnosed as osteofibrous dysplasia. The reason for misdiagnosis is mainly because the disease is rare, and it is very similar to dysplasia of bone fibrosis. It is also related to the progressive development of the characteristics of the disease. Pathological examination is the main method for diagnosis. If pathological fractures are present, biopsy should be performed as soon as possible, and the central part of the tumor should be removed to avoid the influence of fracture repair response on the diagnosis. The pathological diagnosis of recurrent cases should be based on the first biopsy material, and the interference of the postoperative response to the diagnosis should be excluded. When it is difficult to distinguish between dysplasia of bone fibrosis, more points should be taken to understand the whole picture of the tumor, which is mainly determined by the present condition.