A 35-year-old black male with the abrupt onset of blurred vision and decreased visual acuity was found to have severe retinal vasculopathy associated with systemic light chain deposition disease. While ocular manifestations are frequently reported in other plasma cell dyscrasias; ophthalmologic symptoms have not been previously reported in systemic light chain deposition disease alone. A possible mechanism of light chain deposition and damage to the retinal vasculature is proposed.