Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare disease-causing vision loss due to a solid pigmented lesion within the macula. It is a benign condition that can cause significant visual loss and the entity is limited to retinal tissue. The aetiology is unknown, and there is no standard guideline. Patients should have regular eye examinations to monitor changes in the lesion or vision. Hence, we describe a case of 18-year-old female with diminished vision in left eye since childhood and on fundus examination, clear media with size of 4–5-disc diameter elevated subretinal greyish yellow lesion noted in posterior pole and peripapillary area with epiretinal membrane causing apparent traction of retina, with overlying dilated and tortuous vessels. Optical coherence tomography showed highly reflective elevated lesion with hyporeflective shadowing of underlying tissue. Patient was under observation, then discharged with regular follow-up advice.
Keywords: Congenital simple hamartoma of the retinal pigment epithelium, Macula, Visual acuity, Benign, Optical coherence tomography.