Sebaceous carcinoma accounts for 1-5.5% of all eyelid malignancies. This lesion, traditionally considered among the most lethal of all tumors of the ocular adnexa, occurs more commonly in women and in the elderly and has a predilection for the upper lid. Both clinically and histologically, sebaceous carcinoma may masquerade as benign or less invasive conditions such as chalazion, blepharitis, conjunctivitis, meibomitis, superior limbic keratoconjunctivitis, basal cell carcinoma and carcinoma in situ, resulting in delayed diagnosis and treatment. In this review the incidence, clinical presentation, and prognostic factors (both clinical and histopathologic), and treatment modalities are discussed.