Abstract
The Rieger's Syndrome is characterized by hypoplasia of the iris, pupil and dental defect.
In dental defect, it is included malocclusion, partial anodontia, microdontia and abnormally shaped teeth.
This report presented two cases in a family of Rieger's Syndrome.
A 8-year-old girl, one of the two cases, was found to have hypoplasia of the iris, slitlike pupils, progenia, partial anodontia, microdontia and peg-shaped teeth.
39-year-old her mother was found to have abnormally shaped pupils, glaucoma, progenia and partial anodontia.