A case of phakomatous choristoma of the lower eyelid is described. A survey of previously reported cases reveals a characteristic and consistent clinical and morphologic picture. Universally the patient presents in infancy with a rather small, firm, rubbery nodule attached to the lower edge of the inferior tarsal plate. Always present in the nasal aspect of the lower lid, the tumor is easily palpable. Recognition of this clinical picture may allow the ophthalmologist to suspect the appropriate diagnosis, but the final diagnosis should be based on the recognition of characteristic histopathology.