Granulomatous angiitis is a rare idiopathic necrotizing granulomatous vasculitis that often involves multiple systemic organs. Ocular manifestations are uncommon but tend to be severe. The clinical presentations include conjunctivitis, scleritis, peripheral corneal ulceration, uveitis, orbital pseudotumor, lacrimal duct obstruction, dacryocystitis, and peripheral retinal arteritis. Proptosis is observed in 15%–20% of cases, while a few cases present with central retinal artery occlusion and central retinal vein occlusion.