Introduction: Kikuchi Fujimoto is a rare necrotizing lymphadenitis disease. We report a case of a 17-year-old boy, known case of Kikuchi Fujimoto disease (KFD), who developed left eye central retinal vein occlusion (CRVO). A 17-year-old boy presented with left Case Presentation: eye sudden diminution of vision. He was diagnosed with Kikuchi Fujimoto necrotizing lymphadenitis disease 2 years back. He had left eye central retinal vein occlusion with disc and macular edema. Optical coherence tomography (OCT) showed subretinal uid, cystoid macular edema along with disc elevation. He was on s Hydroxychloroquine since 6 months. After systemic evaluation he was started on oral prednisolone 40mg on weekly 10mg tapering dose. After a month his macular edema as well as disc edema had signicantly resolved. KFD is itself is a rare Conclusion: entity and additional manifestation of CRVO in them, makes it rarest of rare presentation. If not diagnosed on time and treated can lead to permanent vision loss