An eight-year-old boy presented with decreased vision in both eyes. At presentation, the visual acuity was 6/60 in both eyes with high plus spheres. Anterior segment examination was normal. Fundus examination and spectral domain optical coherence tomography were consistent with posterior microphthalmos and showed an elevated foveal contour and fold in the outer plexiform layer. External limiting membrane, photoreceptor and retinal pigment epithelium were not involved in the fold. To the best of our knowledge this is the first such case report with optical coherence tomography imaging of the retinal layer involved in a case of posterior microphthalmos.