A 12-year-old patient presenting with absence of the optic disc and retinal blood vessels associated with atrophic macular lesion was studied. A toxoplasma IgG antibody test had been found positive at the age of 4 months. This test and the clinical findings suggested the possibility of optic nerve aplasia with atypical macular coloboma due to congenital toxoplasmosis. The pro's and contra's concerning this hypothesis are discussed by the authors.