To report a case of pleomorphic adenoma arising in the ectopic lacrimal gland of the lower eyelid.

A 36-year-old male presented with a gradually increasing mass in the left lower eyelid that had been growing for one year. A hard, non-tender, subcutaneous mass was palpated on the lateral one-third of the left lower eyelid, and there were no clinically specific signs. Orbital computed tomography demonstrated a well-demarcated 1 × 0.7 × 0.7-cm-sized mass with heterogeneous enhancement, and complete surgical resection was performed. The mass was non-adjacent to the conjunctiva and inferior tarsal plate. Histological examination showed glandular elements embedded in a myxoid stroma. The mass was diagnosed as pleomorphic adenoma arising in the ectopic lacrimal gland. At the postoperative 6-month follow-up, there was no recurrence or abnormal finding at the operation site.

Pleomorphic adenoma arising in the ectopic lacrimal gland should be considered as a differential diagnosis of eyelid masses.