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The occurrence of an accessory palpebral fissure and eyelid is an extremely rare phenomenon. An isolated accessory palpebral fissure and eyelid have been reported only twice in the literature, and in one case as an extension of Delleman syndrome, or oculocerebrocutaneous syndrome. The authors report a case of a full-term newborn who presented with an accessory palpebral fissure and eyelid associated with microcornea, skin polyps and tags, cutis dysplasia, and hypoplasia of the corpus callosum with an otherwise normal systemic workup and negative genetic screening. Detailed surgical management and histopathological analysis of the accessory findings are also described.