This report describes 12 patients with unilateral peripapillary myelinated nerve fibers associated with myopia and/or amblyopia. Seven patients had myopia with a mean of -13.00 diopters of anisometropia and abnormal maculae varying from a decreased reflex to pigment dispersion. These patients had final visual acuities of 20/200 or less following conventional amblyopia therapy. In contrast, five patients had myopia with a mean of -3.75 diopters of anisometropia and normal maculae. These patients had final visual acuities of 20/30 or greater with identical therapy. There was a statistically significant difference between the mean diopters of anisometropia of these two groups. However, the range of diopters of anisometropia overlapped, and the critical feature that determined which patients could achieve good vision was the macular appearance. This condition is distinct from simple unilateral myopia with amblyopia and from myelinated nerve fibers without myopia or amblyopia.