Purpose To highlight that in retinal racemose hemangioma (RRH) complicated by central retinal vein occlusion (CRVO), intravitreal anti-VEGF may only partially resolve macular edema, and timely laser photocoagulation is the decisive adjunct. Case presentation A 46-year-old female presented with vision loss in the left eye. Fundus examination revealed CRVO and a superotemporal RRH. Optical coherence tomography (OCT) confirmed significant macular edema. After three monthly anti-VEGF injections, hemorrhages subsided partly but macular edema persisted. Subsequently, laser photocoagulation was applied to retinal non-perfusion areas along with continued anti-VEGF therapy, and at 4 months the macular edema had completely resolved, accompanied by a marked improvement in visual acuity. At each subsequent follow-up visit, the patient’s ocular status stayed stable, with no evidence of recurrent macular edema. Conclusion In RRH-associated CRVO, although anti-VEGF therapy effectively managed the acute macular edema, complete resolution required adjunctive laser photocoagulation.