An 11-year-old Japanese girl had a greenish-gray, slightly elevated lesion at the optic disc and juxtapapillary retina of the right eye that was diagnosed as a combined hamartoma of the retina and retinal pigment epithelium. A gliotic epiretinal membrane partially covered the lesion. Tortuous retinal vessels, which leaked fluorescein dye, were noted ophthalmoscopically. The lesion remained stable during a follow-up period of 1 year. After reviewing the Japanese literature, we concluded that the combined hamartoma may not be as rare in Japan as previously thought.