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Phakomatous choristoma is a rare congenital hamartoma of lens tissue. It presents in newborns or young infants as a subcutaneous mass in the medial lower eyelid. We present a case of phakomatous choristoma recently identified among old consultation files at Children's Hospital in Boston. Our case illustrates unique histological features of this entity which has to be included in differential diagnosis of skin lesions of the inferonasal eyelid.