BACKGROUND Sickle cell disease (SCD) is a hereditary hemoglobinopathy, which leads to microcirculatory disturbances of various organ systems through recurrent vaso-occlusive episodes, with a possibly fatal outcome. Sickle cell retinopathy (SCR) is the best described ocular manifestation of SCD. Irrespective of the presence of peripheral SCR, sickle cell maculopathy (SCM) can occur early in the course of the disease. METHODS Review of the international and German literature on ocular involvement in SCD with a focus on SCR and SCM and an overview of current systemic therapeutic approaches in SCD on the occasion of the presentation of two patients with HbSS SCD. CONCLUSIONS In contrast to SCR, SCM with temporal thinning of the inner retinal layers has only been increasingly described in the literature in the last 5 years, with the advent of SD-OCT and OCTA. Irrespective of the presence of SCR, as many as about half of the patients may develop SCM early in the course of the disease. As a result of progress in systemic therapeutic options and due to migration, the clinical picture will occur more often also in Germany. By knowing about this complication of SCD an early diagnosis can be made and unnecessary diagnostics can be avoided.