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