Purpose: To report a 4-year-old boy with a large, macula-involving combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) lesion with an associated choroidal neovascular membrane involving the fovea, characterized with multimodal imaging. Methods: Case report. Results: Given the low likelihood of visual improvement with intervention, observation was recommended and the CHRRPE remained stable on follow-up 4 months after presentation. Conclusion: CHRRPE is a rare congenital retinal lesion that is variably pigmented. Awareness of rare complications, such as CNVM, as seen in this pediatric case is paramount.