Intraretinal myelination of ganglion cell axons occurs in about 1% of humans and when observed ophthalmoscopically, appears as a white or opaque patch within the fiber layer. Previous studies of myelinated retinal tissue have largely been conducted at the light microscopic level. Three retinae with intraretinal myelination and one normal retina were obtained post-mortem and prepared for electron microscopy. The present study showed that myelinated patches in the human retina contained a mixture of unmyelinated and myelinated axons. Within this population of myelinated axons were structures which were abnormal and there were obvious signs of axonal and myelin sheath degeneration within the myelinated patches. Outside these myelin patches the retina appeared normal without signs of degeneration indicating that post-mortem degeneration prior to fixation could not account for all of the degenerative changes observed. The lack of significant numbers of macrophages and lymphocytes indicated that there was no concomitant inflammatory process within the myelin patches. The myelination present within these eyes appeared to be due to the anomalous location of oligodendrocytes. Both unmyelinated and myelinated axons had larger diameter than axons measured within normal areas of the retina or those within the optic nerve.