A 10 year old male patient presented with diminished vision in his right eye since 6 years. He had not taken any treatment for the same. On examination visual acuity in the right eye was 20/720 and left eye was 20/20. Anterior segment was normal in both eyes. Fundus examination of the right eye showed an ill-defined pigmented lesion measuring 14 mm × 10 mm in its greatest dimensions. The retina was slightly elevated and there was a greyish appearance of the macular surface suggestive of an epiretinal membrane (ERM). The temporal arcades were distorted and straightened. Ultrasonography demonstrated a plaque like retinal lesion showing high reflectivity. Optical coherence tomography (OCT) showed disorganized retinal architecture and thickening with an overlying ERM. The patient was diagnosed to have combined Hamartoma of Retina and RPE (Figures 1-3).

Figure 1: The ill-defined lesion is seen to extend from 2 Disc diameters (DD) nasal to the disc, to 5 DD temporal to the disc and 2 DD superior and inferior to the disc. The retina appears elevated and has a greenish grey discoloration suggestive of a thick epiretinal membrane. The ERM is causing distortion to the temporal vascular arcades.

Figure 2: B-scan showing a plaque like lesion corresponding to a high reflective spike on a A-scan.

Figure 3: Line scan of OCT of the right eye showing disorganized retinal architecture with thickening and an adherent epiretinal membrane.