Sturge—Weber综合征临床少见,国内报道不多.我们曾遇一典型病例,现报告如下. 患者男,16岁.以面部血管痣、智力障碍、反复抽搐就诊。患者出生时左面部即见一片状葡萄酒色斑,随年龄增长,范围不断扩大.智力发育缓慢,语言不清.6岁后反复出现癫痫样发作及痉挛性偏瘫,近年发作频繁.家族中无此类患者. 体查不合作,答非所问,智力低下.面部及口底、软腭弥散性分布有葡萄酒色斑状血管痣.左上颌骨肥大,上下唇肥厚.上颌牙龈增生,左下肢较右侧 Sturge-Weber syndrome clinical rare, few domestic reports.We have encountered a typical case, are as follows.Patient male, 16 years old.With facial nevus, mental retardation, repeated convulsions. Patient at birth, the left side of the area to see that a piece of wine stain, with age, expanding the scope of mental retardation, language is unclear .6 years after the recurrence of epileptic seizures and spastic hemiplegia in recent years, seizures frequently. Such patients. Physical examination of non-cooperation, non-questioning, mental retardation. Face and mouth at the end of the soft palate diffuse distribution of wine pigmented nevus. Left maxillary hypertrophy, upper and lower lip hypertrophy. Gingival hyperplasia, left lower extremity than the right