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To develop an anatomical classification scheme for combined hamartoma of the retina and retinal pigment epithelium (RPE) and specify recommendations for follow-up interval.Retrospective review of patients with combined hamartoma of the retina and RPE examined during a 7-year period (2008–2015). The clinical presentation, fundus examination, and optical coherence tomography were analyzed.Lesions were classified based on location, fundus features, and optical coherence tomography findings. Lesion location: macular/peripapillary—Zone 1; mid-periphery—Zone 2; and far periphery—Zone 3. Associated fundus findings: no retinal traction—Stage 1; retinal traction and/or retinoschisis—Stage 2; and retinal detachment—Stage 3. Optical coherence tomography findings: epiretinal component only—A; partial retinal involvement—B; and complete retinal and RPE involvement—C. Complete ophthalmologic evaluation is recommended at least every 6 months for patients younger than 12 years, with more frequent follow-up in patients with: lesions in the macula/peripapillary (Zone 1) or with retinal traction, retinoschisis, or retinal detachment (Stage 2 and 3). Surgical intervention is recommended in patients with vision loss secondary to macular traction or retinal detachment.A new clinical classification system is proposed for evaluating and managing patients with combined hamartoma of the retina and RPE. The zone and stage of combined hamartoma of the retina and RPE lesion will assist in determining follow-up interval and surgical intervention. Application of a uniform classification scheme will facilitate assessment and comparison of findings across different studies.