目的：对罕少、疑难的寰椎原发性肿瘤的诊治提出一个总策略。方法和结果：总结手术治疗２例寰椎原发性肿瘤的成功实践，复习国外迄今所报道的共１７例寰椎原发肿瘤的有关文献（国内迄今无报道），分析寰椎原发性肿瘤的发生特点和处理途径。结论：寰椎肿瘤极罕见，易误诊，发生于前弓和侧块的肿瘤处理困难，但及时的手术治疗及必要时结合放疗可以取得较好的效果。 Objective: To propose a general strategy for the diagnosis and treatment of rare and difficult primary tumors of the atlas. Methods and results: Surgical treatment of 2 cases of primary tumors of the sacrum was reviewed. The literature on 17 cases of primary sacral tumors reported so far in foreign countries was reviewed (no domestic reports so far) and the characteristics of primary tumors in the sacrum were analyzed. And processing approaches. Conclusion: The sacral vertebral tumor is extremely rare and misdiagnosed. It is difficult to deal with the tumor in the anterior arch and lateral mass. However, timely surgical treatment and combined radiotherapy may achieve better results.