Background: Trichilemmal carcinoma (TLC) is a rare malignant adnexal tumor predominantly affecting the scalp, eyelids, neck and face of the elderly. Here, we firstly report a rare case of corneal perforation caused by TLC grown in eyelid margin. Case presentation: A 68-year-old female presented with two months history of unprovoked redness, pain and blurred vision in the left eye. On slit-lamp examination, a 1×2mm aseptic corneal perforation embedded by iris prolapsed was noted. After excluding other causes through examinations and case history inquiry, we speculated that the severe MGD and subsequent BKC might be the cause. In order to prevent the ulcer enlargement and intraocular infection, the patient was treated with penetrating keratoplasty immediately. However, a terrible phenomenon has aroused our concern that several small nodules gradually developed on the eyelid margin, accompanied with bleeding, burst and madarosis postoperatiely. The biopsy revealed the eyelid was involved by TLC and then the lesionectomy was performed immediately. There was no evidence of local recurrence and metastasis during 1 year follow-up. Conclusions: The involvement of eyelid margin by TLC, and subsequent BKC and corneal perforation were very rare and it has not been reported yet. The concomitant BKC can be gradually healed after rectifying eyelid margin by total excision of TLC. It is very important to offer careful follow-up to all patients.