Unilateral macular infarction occurred in a 23-year-old black man with sickle cell hemoglobinopathy (SS), who was already receiving oxygen and hydration treatment for a painful crisis. Visual acuity of 20/200-1 in the affected eye did not improve with 36 hours of "conservative" treatment. Fifteen hours after an exchange transfusion, however, visual acuity started to improve. By 36 hours after transfusion, a visual acuity of 20/40 was achieved, with the gradual return over 8 weeks to 20/20. This case provides strong circumstantial support for the effectiveness of exchange transfusion in sickling maculopathy.