To report a case of multiple eyelid trichilemmomas associated with Cowden syndrome.

A 27-year-old woman presented with multiple upper and lower eyelid skin masses that developed over several years. The masses were as large as whitish millet, and were around the upper and lower eyelid margin and the face. The patient had previously undergone subtotal thyroidectomy for a thyroid mass and a mass excision for extremity hemangioma. Excisional biopsy was performed for the diagnosis, and trichilemmoma was diagnosed based on histopathologic examination. Consequently, multiple trichilemmoma associated with Cowden syndrome was diagnosed, and breast evaluations for existence of further masses were recommended. On breast examinations, intraductal papilloma and fibroadenoma were detected.

The trichilemmoma was a hair-follicle benign tumor that also appeared on the skin around the eyelid. If multiple trichilemmoma is diagnosed, an association with Cowden syndrome should be considered as well as presence of masses in other organs.