We report the clinical and angiographic features as well as the natural course of an optic disk and juxtapapillary combined pigment epithelial and retinal hamartoma in a 23-year-old white girl. The patient presented 3 episodes of recurrent vitreous hemorrhages in the first 4 years of follow-up, which were spontaneously and totally absorbed. During the last 6 years, she was absolutely free of any ocular symptom. The tumor did not show any growth during the 10 years of follow-up. The digital indocyanine green angiography, which was normal in the early frames, revealed a mild, patchy hyperfluorescence corresponding to the tumor location in the late phase.