The detailed examination of eye structure and function in numerous Drosophila mutants has provided unprecedented insights into retinal physiology and pathology. Many Drosophila mutants that undergo retinal degeneration (RD) exhibit structural and/or dynamic alterations of the photon receptor rhodopsin, and similar defects cause retinitis pigmentosa (RP) in humans. Here, we consider how the use of Drosophila has advanced our understanding of RP and highlight new mechanisms underlying rhodopsin-mediated pathologies in the retina.