Objective: The aim of the present study was to characterize sickle cell disease retinopathyin children and teenagers from Bahia, the state in northeastern Brazil with the highestincidence and prevalence of sickle cell disease.Methods: A group of 51 sickle cell disease patients (36 hemoglobin SS and 15 hemoglobinSC) with ages ranging from 4 to 18 years was studied. Ophthalmological examinations wereperformed in all patients. Moreover, a fluorescein angiography was also performed in over10-year-old patients.Results: The most common ocular lesions were vascular tortuosity, which was found in nine(25%) hemoglobin SS patients, and black sunburst, in three (20%) hemoglobin SC patients.Peripheral arterial closure was observed in five (13.9%) hemoglobin SS patients and in three(13.3%) hemoglobin SC patients. Arteriovenous anastomoses were present in six (16.5%)hemoglobin SS patients and six (37.5%) hemoglobin SC patients. Neovascularization wasnot identified in any of the patients.Conclusions: This study supports the use of early ophthalmological examinations in youngsickle cell disease patients to prevent the progression of retinopathy to severe disease andfurther blindness.