Trichilemmal carcinomas are rare malignant adnexal tumors which can involve the head and, rarely, the eyelid. Accurate diagnosis and differentiation from basal cell carcinoma or trichoepithelioma are dependent on histopathological and immunohistochemical analysis. We report a case of an eyelid mass, initially diagnosed by incisional biopsy as a trichoepithelioma, later found on excisional biopsy to be a trichilemmal carcinoma and present a review of the literature of the histopathology, differential diagnosis and management. Consideration of these entities will be useful to the histopathologist and clinician to better identify and treat these challenging lesions.