Background:We describe a case where hyperviscosity retinopathy and immunogammopathy maculopathy were the presenting features of IgA multiple myeloma and report the response of maculopathy to intravitreal injection of dexamethasone implants.Case presentation:A 56-year-old man presented at the Department of Ophthalmology with the chief complain of reduced vision for the past 10 days in both eyes. Ophthalmic examination revealed central retinal vein occlusion resembling signs with severe macular edema in both eyes with prominent serous macular detachment. After comprehensive evaluation, an IgA type kappa multiple myeloma was diagnosed complicated with hyperviscosity-associated retinopathy and immunogammopathy maculopathy. Patient was treated with multiple sessions of plasmapheresis, systemic chemotherapy, and finally intravitreal implants of dexamethasone with complete restoration of macular edema and serous macular detachment in both eyes. The visual function and the hyperviscosity-associated retinopathy were partially restored.Conclusion:Ocular manifestation might be the only presenting sign of a life-threatening disease such as IgA multiple myeloma. A high level of suspicion is required to diagnose and treat such cases promptly and effectively.