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To assess the clinical course of choroidal neovascularization (CNV) in patients with angioid streaks using optical coherence tomography and fluorescein angiography/indocyanine green angiography.We examined a consecutive series of 88 eyes of 44 patients with angioid streaks using color fundus photography, optical coherence tomography, and fluorescein angiography/indocyanine green angiography.At the initial visit, 33 eyes exhibited no CNV, 2 exhibited polypoidal choroidal vasculopathy, 8 exhibited Type 1 CNV, 32 exhibited active Type 2 CNV, and 13 exhibited a fibrotic scar. In addition to the 2 eyes that exhibited macular polypoidal choroidal vasculopathy at the initial visit, 3 exhibited peripapillary polypoidal lesions, and 2 exhibited polypoidal lesions at the edge of the preexisting Type 2 CNV/fibrosis. During the follow-up, Type 2 CNV developed in 4 eyes on the basis of Type 1 CNV. Visual acuity was worse in eyes with Type 2 CNV and fibrosis than in those with Type 1 CNV, while polypoidal choroidal vasculopathy did not affect the visual acuity.Eyes with angioid streaks can develop any form of CNV including polypoidal choroidal vasculopathy. Considering the worse visual acuity in eyes with Type 2 CNV and fibrosis, patients should be carefully observed so as to treat them promptly when Type 2 CNV occurred beneath the fovea.