Twenty-two members of two families have been identified as being affected with iridogoniodysgenesis. The major clinical features of this disorder are mesodermal remnants in the iridociliary angle associated with abnormal angle vasculature, marked hypoplasia of the iris stroma and increased intraocular pressure leading to glaucoma. The involvement of the two eyes is remarkably symmetric. Pedigree analysis of the larger family provided firm evidence for regular autosomal dominant inheritance as the mechanism of genetic transmission.