We examined the hospital records (including fundus photographs) of 769 patients (1486 eyes) with sickle cell disease from June 1978 to July 1985 for evidence of epiretinal membranes in the macula (51 eyes) or extramacular region (four eyes) of the posterior pole. The incidence of epiretinal membranes, seen in the eyes of patients with hemoglobin SC, SS, and SB+ thalassemia, was 3.7%. Most epiretinal membranes were mild, and visual acuity was 20/40 or better in 78.2% of these eyes. Severe macular pucker was occasionally noted following retinal detachment surgery. One patient with SC disease developed a macular epiretinal membrane that led to a macular hole and eventually to macular and extra-macular retinal detachment. Epiretinal membranes are a relatively common cause of maculopathy in patients with sickle cell disease.