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Sebaceous gland carcinoma, commonly arises in the periocular area, is an uncommon condition. It represents 1-5.5% of eyelid malignancies and is considered to be the third most common eyelid malignancy after basal cell and squamous cell carcinomas, although few reports placed this tumor as second most common after basal cell carcinoma. It usually affects elderly women and characterized by high rate of local recurrence, regional, and distant metastases. A delay in diagnosis, which can be attributed primarily to ability of this tumor to masquerade as more benign conditions, often leads to inappropriate management with increased morbidity and mortality rates. In this study, the authors discuss key elements of the primary disease and therapeutic options available to treat such devastating problem.