目的:通过对5例颞骨先天性胆脂瘤的资料分析,探讨其误诊原因。方法:5例术前均经影像学检查确诊而行手术治疗。结果:5例均手术治愈,分别随访2～5年,患者全身情况良好。结论:在鼓膜完整情况下,虽有面瘫、外耳道溢液或耳闷痛等主诉,但先天性胆脂瘤常易漏诊和误治。影像学和仔细的局部检查可提高诊疗水平,从而避免严重的并发症发生。 Objective: To investigate the causes of misdiagnosis by analyzing the data of 5 cases of temporal bone congenital cholesteatoma. Methods: All the 5 cases were confirmed by imaging examination before surgery. Results: All the 5 cases were cured by surgery. The patients were followed up for 2 to 5 years respectively and the patients were in good condition. Conclusion: In the erection of the intact tympanic membrane, although facial paralysis, external auditory canal discharge or ear pain and other complaints, but often congenital cholesteatoma misdiagnosis and misdiagnosis. Imaging and careful local examination can improve the level of diagnosis and treatment to avoid serious complications.