A 22-year-old Chinese woman noted a sudden decrease in vision in both eyes 3 days after being histologically proven Kikuchi–Fujimoto disease. Fundus fluorescein angiography of both eyes showed multiple focal areas of retinal arteriolae occlusion with adjacent areas of capillary non-perfusion and leakage from the dilated retinal vessels in the posterior pole and midperiphery. During the follow-up period of 7 months, the patient was treated with intravenous methotrexate and a low maintenance dose of prednisone. Occlusive vasculitis resulted in proliferative retinopathy in both eyes and there were no other systemic abnormal findings. Autoimmune event was considered as the possible pathogenesis of occlusive retinal vasculitis.