We report the case of a 14-year-old boy with history of microangiopathic hemolytic crises secondary to atypical hemolytic uremic syndrome presenting with new-onset decreased vision, flashes, and floaters in his left eye. The patient had a history of chronic retinal detachment in the right eye and retinal neovascularization in the left eye treated with panretinal photocoagulation at age 5. He was now found to have a new combined tractional-rhegmatogenous retinal detachment in the left eye. Despite surgical reattachment of the retina, he had progressive retinal and optic nerve ischemia, with resultant left eye visual acuity of light perception. To our knowledge, this is the first reported case of proliferative retinopathy and tractional and rhegmatogenous retinal detachments in a pediatric patient with atypical hemolytic uremic syndrome.