Clinical findings in aniridia may include corneal epitheliopathy, glaucoma, lens subluxation and cataract, and hypoplasia of the fovea and optic nerve. We report the occurrence of retinal detachment due to giant tears in four eyes of three children with aniridia. All eyes were buphthalmic and none had undergone lens or posterior segment surgery. All operated eyes underwent vitreolensectomy and silicone oil injection; useful vision was restored in two eyes. The pathogenesis and management of this previously unreported complication are discussed.