Iris malformations are often associated with malformations of the entire eye and systemic diseases. Malformations of the anterior chamber angle can lead to juvenile glaucoma. Axenfeld-Rieger syndromes have autosomal dominant transmission and are associated with juvenile glaucoma in 50-60% of patients. Besides eye anomalies craniofacial malformations are also typical. The etiology of iridocorneal endothelial (ICE) syndrome is unclear but atypical endothelial cells lead to malformations of the entire anterior eye segment of the eye and glaucoma. Aniridia is a bilateral, congenital malformation which manifests sporadically and transmission is usually autosomal dominant. Glaucoma, malformations of the entire eye and systemic malformations are associated with aniridia. Conservative and microsurgical treatment of glaucoma of these syndromes need the cooperation of different medical subspecialties and are often ineffective.