Chondroid syringoma is a benign mixed tumor characterized by sweat gland elements in a cartilaginous stroma. This rare tumor accounts for only 0.01% of all primary skin tumors and occurs only rarely in the periorbital region. Usually between 0.5 cm and 3.0 cm, risk of malignancy increases in chondroid syringomas greater than 3.0 cm in size. Here, the authors report a rare case of giant chondroid syringoma arising in the lower eyelid, characterized by keratinized stratified epithelium in a cartilaginous stroma. This case illustrates the importance of considering a possible diagnosis of chondroid syringoma in the evaluation of eyelid masses.