Rieger 氏综合征为眼前节中胚层发育异常伴外胚层组织发育不良的一组综合征。本征早期手术疗效较好,今将我科所见1例报告如下。患者王××女22岁1991年8月13日入院,自幼双眼视物不清,逐渐加重。左眼向前突,已失明3年。患者足月顺产,家族中无相同眼病。发育营养一般。头略小、下颌略短。全牙裂反(牙合)、腭弓高,牙齿稀疏,前牙散在间隙(图1)。心、肺等其他正常。血压120/85mmg。血、尿常规及肝功正常。X 线全口曲面断层见上、下颌骨发育不良,缺牙:(?)缺牙处无牙胚。(?)牙根发育不良。视力右眼0.01(— Rieger’s syndrome is an anterior segment mesoderm dysplasia with ectodermal dysplasia in a group of syndromes. Efficacy of early surgery is better, we will see a case of this report is as follows. Wang XX female patient 22 years old admitted to hospital on August 13, 1991, his eyes blurred, gradually increased. Left eye forward sudden, has been blind for 3 years. Full-term follow-up of patients with the same family, no eye disease. Developmental nutrition in general. Head slightly smaller, slightly shorter jaw. Tooth split anti (occlusion), palatal arch high, sparse teeth, anterior teeth scattered in the gap (Figure 1). Heart, lung and other normal. Blood pressure 120 / 85mmg. Blood, urine and liver function normal. X-ray full mouth curved surface see, mandibular dysplasia, lack of teeth: (?) Edentulous at no tooth germs. (?) Root dysplasia. Right eye 0.01 (-