目的　阐述先天性虹膜基质囊肿的临床特点、病理组织学特点以及有效的治疗方法。方法　对我院 1 979年～ 2 0 0 3年诊治的 8例先天性虹膜基质囊肿的临床资料进行回顾性分析。结果　 7例为婴幼儿或青少年 ( 5个月～ 1 9岁 ) ,1例为 4 8岁成年人。确诊年龄 :6例在 1岁前确诊 ,2例分别为 1 0岁和 2 0岁。女 7例 ,男 1例。左眼 7例 ,右眼 1例。位置 :虹膜下方 5例 ,虹膜颞侧 1例 ,累及全部虹膜 2例。 5例术前眼压高 ( 2 6～ 6 4mmHg)。 8例均有角膜混浊、变性。 3例晶状体混浊。本组 7例均采用囊肿抽吸联合扇形或全部虹膜囊肿切除 ,术后无复发。 1例眼球摘除。结论　先天性虹膜基质囊肿主要发生于婴幼儿。早期采用囊肿抽吸联合包括囊肿在内的扇形或全部虹膜切除是最有效的治疗方法。 Objective To describe the clinical features, histopathological features and effective treatment of congenital iris stromal cyst. Methods The clinical data of 8 cases of congenital iris stromal cysts treated in our hospital from 1979 to 2003 were analyzed retrospectively. Results Seven cases were infants or adolescents (5 months to 19 years) and one case was 48 years old. The diagnosis of age: 6 cases were diagnosed before the age of 1, two cases were 10 years old and 20 years old. Female 7 cases, 1 male. Left eye in 7 cases, right eye in 1 case. Location: Under the iris in 5 cases, iris temporal side in 1 case, involving all of the iris in 2 cases. Five patients had high intraocular pressure (26 ~ 64 mmHg). All 8 cases had corneal opacity and degeneration. 3 cases of lens opacity. The group of 7 patients were treated with cyst suction fan or all iris cyst resection, no recurrence. 1 case of enucleation. Conclusions Congenital iris stromal cyst occurs mainly in infants. Early use of cyst sucking, including cysts, including scalloped or total iris resection is the most effective treatment.