Abstract: The intersection of retinal and neurodegenerative disease has drawn increasing attention, revealing the retina not only as a passive bystander but also as an active participant in central nervous system (CNS) pathology. Tauopathies such as Alzheimer’s disease (AD) and frontotemporal dementia are characterized by intracellular tau accumulation and synaptic dysfunction—hallmarks that have been observed in the retina of patients and animal models. Amyloid-beta (Aβ) pathology, another defining feature of AD, has similarly been detected in retinal tissues (Gupta et al., 2021; Gaire et al., 2024; Davis et al., 2025). This convergence supports a growing paradigm in which the retina serves as both a surrogate marker and a mechanistic substrate for CNS disease progression. Induced pluripotent stem cell (iPSC)-derived retinal systems, including 2D retinal neurons, 3D retinal organoids, and iPSC-derived retinal pigment epithelium, are redefining how we model neurodegeneration. These human-based platforms offer direct access to disease-relevant phenotypes, genetic precision, and the possibility of patient-specific studies. This article explores how these models illuminate taudriven pathology, enable mechanistic dissection, and hold promise for therapeutic and biomarker development in neurodegenerative and retinal diseases.