A 4-year-old male Labrador retriever mix presented with blepharospasm and elevated intraocular pressure in the right eye. Ophthalmic examination revealed signs of glaucoma in the right eye including corneal edema, posterior synechiae, and cupped optic disc. The owners elected to enucleate the right globe. Histopathology identified goniodysgenesis, iridocorneal angle closure, retinal degeneration, and corneal edema. Goniodysgenesis is a congenital anomaly involving abnormal development of the iridocorneal angle, which predisposed the dog to glaucoma.