Myelinated retinal nerve fibers are developmental anomalies that are present in approximately 1% of all eyes. They may be associated with ipsilateral high myopia and amblyopia, and also may occur in association with various ocular and systemic abnormalities. Lesions may be congenital or acquired, static or dynamic, and can regress in the setting of several described conditions. The pathogenesis is thought to be due to the presence of ectopic oligodendrocyte-like cells in the retina as a result of a development or acquired insult. Visual acuity is typically poor to begin with, and the improvement of visual function in patients undergoing occlusion therapy has been, in general, limited. We discuss a series of 11 patients seen at the Cole Eye Institute with the condition of myelinated retinal nerve fibers, myopia, and amblyopia and describe the clinical characteristics. Three patients also had optic nerve dysplasia and a discussion of the literature related to this association was also performed.