A 41-year-old woman presented with a rapidly growing mass on her upper left eyelid that had first appeared 3 months earlier. The mass was dark red in color, alternating with whitish pseudocyst formations, and measured 3 cm long with a 1-cm base presenting. The rapid growth had caused a mechanical ptosis and bleeding as the result of erosion of the skin covering the tumor. The initial clinical diagnosis suggested a malignant lesion or vascular tumor; excisional biopsy was performed. The eyelid crease was approached for incision and resection as for a blepharoplasty. Pathologic examination yielded a diagnosis of giant pilomatrixoma. Pilomatrixomas are rare in adults and rarely attain such a large size. After 1 year of follow-up, no recurrence of the tumor has been observed.