This article documents the eighth reported case (to our knowledge) of a phakomatous choristoma, a rare congenital tumor of the lower eyelid of infants. Among the eight cases, there was no instance of tumor recurrence recorded nor was there any detectable postoperative eye defect, despite incomplete resection of the tumor in two patients. While usually clinically diagnosed as a dermoid cyst, the highly characteristic histologic features of this entity should allow more frequent recognition. Ultrastructural examination in our case provided additional evidence of its lenticular origin.
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