目的　探讨积水型无脑畸形的病因、病理及CT诊断与鉴别诊断。方法　本文对 9例积水型无脑畸形患者的临床与CT资料进行了回顾性分析。结果　 9例CT扫描均表现为幕上双侧大脑半球及脑室绝大部分缺失 ,仅见少许残存的枕、额和 /或颞叶组织 ,幕下结构正常。结论　CT扫描直接全面地显示积水型无脑畸形的表现 ,对本病的诊断具有很大的价值。 Objective To investigate the etiology, pathology, CT diagnosis and differential diagnosis of hydrocephalus amebocyte. Methods The clinical and CT data of 9 patients with hydrocephalus without brain deformity were retrospectively analyzed. Results The CT scans of 9 cases all showed the vast majority of cerebral hemispheres and ventricles in the supratentorial area, with only a few residual occipital, frontal and / or temporal lobe tissues and normal infratentorial structure. Conclusion CT scan directly shows the performance of hydrocephalus amebocyte, the diagnosis of the disease has great value.