Granulomatosis with polyangiitis (GPA) is a chronic systemic disease that is characterized by granulomatous inflammation of the upper and lower respiratory tract accompanied by systemic vasculitis of small and medium-sized vessels and necrotizing glomerulonephritis. The etiology is unknown and it is more common in men. GPA is diagnosed with the clinical findings, positive antineutrophil cytoplasmic antibody (c-ANCA), and histological findings. Eye involvement is frequent but retinal vasculitis has rarely been described. Herein we will present a 76-year-old female patient with a GPA diagnosis who was on routine hemodialysis because of renal failure and developed retinal vein occlusion during the clinical follow-up.