SUMMARY

The Axenfeld-Rieger syndrome mesodermal dysgenesis represents a spectrum of developmental disorders characterized by abnormalities of the peripheral cornea, íris and anterior chamber angle associated with developmental defects of the teeth and facial banes. Sparse descriptions of fundus abnormalities have been made, but none have been well documented. We report, to our knowledge for the first time, a case of Axenfeld-Rieger syndrome associated with bilateral "bull's eye" maculopathy.

Key words:
Axenfeld-Rieger; "bull's eye"; maculopathy; glaucoma