PURPOSE:To study the retinal pathologic changes and pathogenesis of relinhis pigmenlosa(RP). METHODS:The relina from a patient with autosomal dominant RP was examined by light and electron microscopy. RESULTS:Degeneration and structure disturbance almost involved in every layer of retina and were accompanied hy regional differenecs:Posterior region was more than periphery one in severity. Degeneration of retinal pigment epithelium(RPE)closely eorrelaled to that of the phmoreceplor. The uhraslrneture of the retina showed extensive and severe degeneration in the photoreeeptors ,particularly ill omer segments and mitoehondrlas. Lipofusein gramdes were accumuhtted in the cytoplagm. CONClUSIONS:These changes suggested that self-energizing system and self engulfing system of the photoreceptols were disfunctloned. （Chin J Ocul Fundus Dis,1997,13: 24-26）