Aim: To present a case of hamartoma of the optic disc and Retinal Pigment Epithelium (RPE) and follow up of the visual function over three-years period. Methods: A seventeen-year-old boy has observed reduced visual acuity in his left eye. The visual acuity was 0.2 and there was RAPD in the left eye. Fundoscopy revealed an elevation of the optic disc obscuring disc vessels with epiretinal gliosis. Fluorescein angiography demonstrated hyperfluorescent saccular dilatations with leakage in the late phase. Ocular Coherence Tomography (OCT) showed hyperreflective elevation of the optic disc and epiretinal membrane. There was a juxtapapillary scotoma in Semi-automated Kinetic Perimetry (SKP). There was no systemic diseases. Results of blood tests, CT and MRI of CNV were normal. Results: After 3 years period of the follow-up the visual acuity was 0.1 and there was a progression of the visual field defect to the altitudinal scotoma in the upper hemisphere. Fluorescein anhiography and OCT revealed the same. Conclusion: Hamartoma of RPE and optic disc is a rare condition consisting of glial, vascular and RPE cells. It should be differentiated from optic disc oedema and vascular tumors of the retina. Assessment of the visual function is very important in the longitudinal follow-up.