Background: Trichofolliculoma of the eyelid and periorbital region remains is rare, and there is limited evidence regarding surgical management, clinical and histological margins, and recurrence rate. Objectives: We performed a systematic review of cases to date on diagnosis and management outcomes of eyelid trichofolliculoma. Methods: Electronic databases were searched for cases reported on trichofolliculoma of the eyelid. The authors extracted and collated data from these studies, and outcomes were summarized descriptively. Results: One hundred twenty citations were identified from database searching. The authors also report a case of eyelid trichofolliculoma. Fifteen studies reporting 19 cases of eyelid trichofolliculoma were finally included. Eighteen out of 19 (94.7%) cases were treated with surgical excision. Of 5 cases which were followed up over an average of 2 years, there was only 1 case of recurrence requiring wedge resection. Conclusions: Recurrence rate in patients with eyelid trichofolliculoma excised with satisfactory margins appears to be low, and no cases of malignant transformation have been reported.