Foveal retinoschisis is rarely found in women. An 11-year-old girl, from non-consanguineous parents, presented with bilateral visual loss from isolated foveal retinoschisis as confirmed by a normal fluorescein angiogram and characteristic optical coherence tomogram. Psychophysical and electrophysiological studies demonstrated mild contrast sensitivity loss, dyschromatopsia and normal full field electroretinographic responses. Visual acuity, foveal retinoschisis, electroretinography, electro-oculography and visual evoked responses remained stable after 13 years but a reduction in pattern electroretinography amplitude was noted. No mutation was found in the coding regions of the RS1 gene. Isolated foveal retinoschisis may be a form of macular dystrophy. Longer-term follow up may contribute to our understanding of this rare disease.