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Purpose: To emphasize the importance of the association of optic disc drusen (ODD) with angioid streaks (AS). Methods: Three patients with ODD and AS underwent a thorough ophthalmic examination including perimetry and B-scan ultrasonography. Fundus fluorescein angiography was performed when indicated. Results: Bilateral ODD associated with AS were present in all of the cases. Pseudoxanthoma elasticum was the definite etiology of the AS in two cases, one of which was complicated by subretinal neovascularization. Laser photocoagulation resulted in closure of the membrane. Conclusions: The follow-up of patients with ODD with regard to the development of AS is essential as ODD may be the earliest clinical manifestation in patients with pseudoxanthoma elasticum, which has potential vision- and life-threatening complications. [ABSTRACT FROM AUTHOR]