Clinical findings of 18-year old female case admitting with the complaint of blindness in her left eye that was present since his childhood were evaluated. Her visual acuities were complete on the right and absolute on the left. Her biomicroscopical anterior segment findings were within normal limits. In her fundoscopic examination, while the findings of the right eye were within normal limits, there were prepapillary elevated glial tissue that was cover the entire papilla, nasally located peripapillary pigmentous changes, and increased number of retinal vessels arising from the disc margin in her left eye. Her left fovea could not be observed. In optical coherence tomography investigation of the optic nerve head in her left eye, there was hyper reflectivity of the prepapillary glial tissue. In the left macular OCT examination, macular thickness was thinner than the other healthy eye and there was no foveal depression. While the cranial and orbital magnetic resonance imagings of the case were within normal limits, her orbital computed tomography and ultrasonography examination disclosed the opacity correlating with calcification in the distal optic disc region.

Keywords: Blindness, congenital abnormalities, optic nerve; optical coherence tomography