Autophagy, a highly conserved self-degradation process that occurs under both physiological and pathological conditions, provides the raw material and energy for cell regeneration under normal circumstances. Dysregulated autophagy under diseased conditions may cause protein accumulation, organelle dysfunction, and even cell death. Recent studies have shown that autophagy regulates the structural integrity and physiological functions of retinal photoreceptor cells and contributes to the pathogenesis of retinopathies such as retinal detachment, age-related macular degeneration, retinitis pigmentosa, and Leber's congenital amaurosis. In this review, we discuss the role of autophagy in photoreceptor cell survival and death in retinal physiology and diseases, and suggest the possibility that autophagy-targeting therapy may be a new strategy for retinal diseases marked by photoreceptor cell death.