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Divided nevus of the eyelid as a rare pathologic entity and develops during fetal growth, when the two eyelids are still fused together. Its surgical treatment is mostly based on full thickness skin grafts. We report a case of a 22-year-old woman with a congenital divided nevus affecting the left upper and lower eyelid. The patient was treated in our Department because of the onset of a purple red nodule in the lower part of the nevus. We examined the case from embryological, histopathological, clinical, and diagnostic standpoints, and discuss surgical options in order to reach an optimal functional and aestetic result.
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