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A history of the 2 syndromes, acute posterior multifocal placoid pigment epitheliopathy and geographic helicoid peripapillary choroidopathy is presented. A complete review of the literature is beyond the scope of this presentation, but the major contributions in recent years are cited. A more complete bibliography may be obtained from our sources. Four cases are presented, each of which fulfills some of the criteria for both syndromes. It is hypothesized that both entities may be variants of one disorder with an obscure etiology.