颅底畸形较为少见，其发生可能与胚胎发育过程中鳃裂未闭有关。多发生于前颅底和恻颅底。临床上分显性与隐性两种，其主要区别为前者常伴有外部畸形，可影响容貌，而后者无外部畸形，常以出现并发症（如反复发作的脑膜炎、脑脊液鼻漏）而就诊。隐性颅底畸形诊断较难，该文介绍 Skull base deformity is relatively rare, its occurrence may be related to the process of embryonic development of the branchial fissure. Occurred in the anterior skull base and skull base. Clinically divided between dominant and recessive, the main difference is that the former often accompanied by external deformity, can affect the appearance, and the latter without external deformities, often with complications (such as recurrent meningitis, cerebrospinal rhinorrhea) and Visit. Diagnosis of recessive skull base deformity more difficult, the article introduced