目的探讨颅面血管瘤病的临床病理特征、诊断与鉴别诊断、治疗及预后。方法回顾性分析1例颅面血管瘤病的临床资料、组织病理学形态。结果光镜下大脑组织灰质萎缩,神经细胞变性、缺失,脑实质内散在较多大小不等的钙化灶,钙化小体呈圆形、同心环状或不规则形,或融合,部分区域见弥漫的钙化小球。局部软脑膜表面可见异常增生的毛细血管网,毛细血管扩张、腔内充满红细胞,呈海绵状血管瘤改变,局部见少量厚壁肌型静脉样血管,间质有出血。结论颅面血管瘤病是一种罕见的先天性神经皮肤综合征,先天性脑部和三叉神经分布区同时有血管瘤存在。临床以颜面血管瘤、癫痫发作为主要症状。 Objective To investigate the clinicopathological features, diagnosis and differential diagnosis, treatment and prognosis of craniofacial hemangiomas. Methods A retrospective analysis of 1 case of craniofacial hemangioma clinical data, histopathology. Results Under the light microscope, gray matter atrophy of brain tissue, degeneration and deletion of nerve cells, scattered calcification in calcification, calcified bodies were round, concentric ring or irregular shape, or fusion, some areas were diffuse Calcified pellets. Local pia mater surface shows abnormal proliferation of capillary network, telangiectasia, cavity filled with red blood cells, cavernous hemangioma change, see a small number of thick-walled muscular venous blood vessels, interstitial bleeding. Conclusions Craniofacial hemangiomatosis is a rare congenital neurodermatosis with concomitant hemangiomas in the congenital brain and trigeminal nerve. Clinical to facial hemangioma, seizures as the main symptom.