Sturge-Weber氏综合征甚为少见。主要病理表现是颅内血管畸形,颜面三叉神经分布区血管瘤,有时还有眼部症状。近年来研究指出本病是神经系统遗传性疾病,为常染色体显性遗传,如D组与G组一个染色体互换位置可引起本病。我院曾遇见一例,现报告如下: Sturge-Weber’s syndrome is rare. The main pathological manifestations of intracranial vascular malformations, facial trigeminal nerve distribution of hemangiomas, and sometimes ocular symptoms. In recent years, studies have shown that the disease is a genetic disease of the nervous system, autosomal dominant inheritance, such as D and G group a chromosomal swap position can cause the disease. Our hospital has met a case, report as follows now: