Sebaceous carcinoma (SbC) is a rare malignant and potentially lethal tumor derived from the adnexal epithelium of sebaceous glands. It represents 5% of all malignant neoplasms in the periocular region and typically presents in the sixth to eight decades of life. The clinical appearance is very diverse, mimicking common ocular benign eyelid inflammations and lesions such as chalazion, blepharitis, sarcoidosis, cutaneous horn, and ocular pemphigoid. Two-thirds of cases are initially misdiagnosed as benign lesions. This tumor can also be mistaken for other more common malignant tumors. Clinically, SbC often appears as a unilateral, firm, indurated, yellow, flesh-colored, or pink mass on the tarsal conjunctiva, caruncle, or eyelid margin, frequently associated with loss of eyelashes and ulceration. Treatment is with wide local surgical resection followed by eyelid reconstruction.