Purpose: We report a case of polycythemia with an ocular complication of branch retinal vein occlusion associated with macular edema that was managed by anti-vascular endothelial growth factor (VEGF) and systemic management. Methods: A 43-year-old, one-eyed male, a known case of polycythemia presented with complaints of decreased vision in the right eye. He underwent comprehensive eye evaluation, fundus photography and optical coherence tomography at the baseline visit and post intravitreal ranibizumab 1-, 3-, 4- and 11-month follow-up. Results: A patient with polycythaemia was diagnosed in the right eye with superotemporal branch retinal vein occlusion and macular edema, which clinically and on optical coherence tomography resolved after 1 intravitreal injection of ranibizumab. However, as he discontinued systemic management, macular edema reappeared and the edema resolved well after intravitreal ranibizumab. He then became more compliant to the systemic therapy and was asymptomatic for the last 7 months. Conclusion: In a case of retinal vein occlusion with macular edema of recognizable cause, the management of systemic disease and anti-VEGF can give satisfactory results.