Rheumatic diseases are not commonly associated with cytomegalovirus (CMV) retinitis. We report a case of bilateral CMV retinitis in a human immunodeficiency virus-seronegative patient with systemic lupus erythematosus (SLE) who was undergoing hemodialysis for end-stage renal disease. The CMV retinitis in this patient was associated with combined azathioprine and low-dose corticosteroid therapy for lupus flare. This association may have important clinical implications because this drug combination is used routinely to treat active SLE. Our patient responded to discontinuation of azathioprine, reduction of the corticosteroid dose, and systemic administration of ganciclovir. We recommend that clinicians maintain heightened awareness of the possibility of CMV retinitis in patients with SLE and end-stage renal disease who are receiving azathioprine and low-dose corticosteroids.