长骨造釉细胞瘤是一低度恶性骨肿瘤，临床上罕见，极易误、漏诊，而失去早期保肢治疗机会．为提高对本病认识，本文将所遇病例，结合文献，讨论其临床病理特征．特征：本瘤好发于３０岁左右人群的胫骨骨干或骨骺偏干部，男性稍多于女性；临床上常有外伤史，局部有隐痛的骨性隆起；Ｘ线征显示骨干部蜂窝状或膨胀性溶骨灶影，无明显骨膜反应，常误诊良性骨病变；病理学检查是确诊的重要手段，特别手术中取物送快速病理检验，对决定手术方案有特殊意义．其病理形态似颌骨造釉细胞瘤，确诊前应排除转移肿瘤，骨及软组织肉瘤；只要对本病提高警觉，早期诊断并不难。早期治疗宜局部扩大切除保肢治疗，局部刮除易复发，本病亦有远处及淋巴道转移可能，故对中晚期和多次刮剔治疗后复发病例，仍需作截肢治疗。 Long bone ameloblastoma is a low-grade malignant bone tumor. It is rarely seen in clinical practice. It is easily misdiagnosed and missed diagnosis. It loses the opportunity of early limb salvage treatment. In order to improve the understanding of the disease, this article will encounter cases, combined with literature, discuss its clinical pathological features. Features: The tumor occurs in the 30-year-old population of the humerus or bone palpebral part of the cadula, males slightly more than women; clinical often have a history of trauma, local pain bulging bone bulge; X-ray sign showing the backbone of the honeycomb or swelling Osteolytic lesions, no obvious periosteal reaction, often misdiagnosed benign bone lesions; pathological examination is an important means of diagnosis, special surgery to take a quick pathological examination, have a special significance for the decision of the surgical plan. The pathological morphology resembles jaw ameloblastoma. Before diagnosis, metastatic tumor, bone and soft tissue sarcoma should be excluded. As long as the disease is alert, early diagnosis is not difficult. Early treatment should be a partial expansion of limb salvage therapy, local scraping is easy to relapse, the disease also has distant and lymphatic metastasis may be, so for the relapsed cases after middle and late and multiple scratch treatment, still need to amputation treatment.