To report a rare case of giant pleomorphic adenoma of the lacrimal gland involving the palpebral lobe causing severe mechanical ptosis and visual field defect, which was removed successfully.

An 82-year-old man visited our clinic with a large protruding mass on his left upper lid that had grown for approximately 10 years causing severe ptosis. Preoperative orbital CT showed a 2.3 × 2.0 cm sized large mass which was suspicious of hemangioma. The mass was completely excised through a lid crease incision. The histopathologic examination showed typical findings of pleomorphic adenoma. After the surgery, preoperative mechanical ptosis was completely resolved and during the 6 months of follow-up, there was no abnormality of tear secretion and evidence of tumor recurrence.

The authors experienced a rare case of giant pleomorphic adenoma of the lacrimal gland involving the palpebral lobe and resolved mechanical ptosis by complete removal of the tumor through a lid crease incision.