Combined hamartoma of the retina and retinal pigment epithelium (RPE) is a rare congenital benign lesion. It is most often detected in young adults and adolescents. The disorder is mostly asymptomatic, however, in 24% of patients the loss of visual function results from complications, such as epiretinal fibrosis associated with tractional distortion of the fovea, hemophthalmos, choroidal neovascularization, exudative retinal detachment, macular edema, and combined tractional/rhegmatogenous retinal detachment. Currently, there is no consensus on the combined hamartoma complications management. The reported clinical case demonstrates the feasibility of staged approach to treatment of combined hamartoma of the retina and RPE complicated by epiretinal fibrosis and partial hemophthalmos using the advanced diagnosis (optical coherence tomography) and treatment methods.