Abstract:  Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) is a rare benign lesion in the macula, parapapillary, or peripheral retina that is commonly found in children. Clinical characteristics were elevation in retina，diferent degree of hyperpigmentation，retinal vascular tortuosity，as well as epiretinal membrane formation. Histopathologically, CHRRPE consists of disorganized glial tissue intermixed with numerous blood vessels and proliferating retinal pigment epithelium cells. Optical coherence tomography is a helpful tool in the diagnosis and management of CHRRPE. Pars plana vitrectomy with membrane peeling may result in restored retinal architecture to some extent and improved or stabled visual acuity for patients with CHRRPE.