Purpose: We discuss a case of macular edema and retinal hemorrhage associated with scleromyxedema. Methods: A case report is presented. Results: A 64-year-old man with history of deep vein thrombosis and pulmonary embolism presented with new-onset rash in the setting of switching anticoagulation treatments. He developed blurred vision, was found to have macular edema and dot blot retinal hemorrhages that improved with systemic and topical corticosteroids. Conclusion: Systemic autoimmune conditions including scleromyxedema should be considered in the workup of occult cystoid macular edema.