Four patients had extensive unilateral myelinated nerve fibers associated with ipsilateral myopia, amblyopia, and strabismus. Their profound visual impairment, exotropia, and the early age onset of symptoms indicated that the amblyopia may have been organically caused. Prognosis is poor for even partial correction but good results have been obtained with intense therapy that includes full correction of the refractive error in each eye and extraocular muscle surgery if cosmetically necessary. In patients with axial myopia, images of a similar size will be produced by placing a lens of the correct power at the spectacle plane. In younger patients, a contact lens should not be used to correct the refractive error because it creates an undesirable anisometropia. Younger patients who have parafoveal fixation and no strabismus respond best to amblyopia therapy.