Sebaceous carcinoma (SC) is a rare tumor with a high rate of local recurrence and metastasis to lymph nodes and organs. The majority of SCs occur in the periocular region frequently presenting as painless, round subcutaneous nodules with a high tendency of diffuse and invasive growth in the eyelid and conjunctiva. It frequently masquerades as inflammatory conditions or as other tumors leading to delay in diagnosis, inappropriate treatment and increased morbidity and mortality. Sebaceous carcinoma is associated with Muir-Torre syndrome, a genetic condition presenting with sebaceous skin tumors associated with internal malignancy. Therefore, SC patients must be carefully evaluated and referred to an internist or gastroenterologist when indicated. Surgery is the definitive therapy for SC. In recent years, less radical surgical strategies are being used with improved outcomes. Current studies demonstrate that Mohs micrographic surgery (MMS) provides maximal tissue conservation and lower recurrence rates. Greater awareness and understanding of SC and its behavior has led to earlier diagnosis and appropriate treatment.