A 12-year-old patient presenting with absence of the optic disc and re
tinal blood vessels associated with atrophic macular lesion was studie
d. A toxoplasma IgG antibody test had been found positive at the age o
f 4 months. This test and the clinial findings suggested the possibili
ty of optic nerve aplasia with atypical macular coloboma due to congen
ital toxoplasmosis. The pro's and contra's concerning this hypothesis
are discussed by the authors.