Diseases leading to retinal cell loss can cause severe visual impairment and blindness. The lack of effective therapies to address retinal cell loss and the absence of intrinsic regeneration in the human retina leads to an irreversible pathological condition. Progress in recent years in the generation of human three-dimensional (3D) retinal organoids (RO) from pluripotent stem cells makes it possible to recreate the cytoarchitecture and associated cell-cell interactions of the human retina in remarkable detail. These human 3D RO systems made of distinct retinal cell types and possessing contextual physiological responses allow the study of human retina development and retinal disease (RD) pathology in a way animal model and two-dimensional cell cultures were unable to achieve. In this review we describe the derivation of ROs from human pluripotent stem cells and their application for modelling RD pathologies while outlining the opportunities and challenges for its application in academia and industry.Copyright © 2023 Elsevier Inc. All rights reserved.