Background: Sebaceous carcinoma is an uncommon adnexal neoplasm with a documented capability for regional and distant metastasis. Traditionally, sebaceous carcinoma has been associated with high rates of recurrence after excision. Objective: To review the current literature on sebaceous carcinoma and detail its epidemiology, pathogenesis, clinical presentation, histopathology, diagnostic workup, treatment, and prognosis. Materials and Methods: Literature review using PubMed search for articles related to sebaceous carcinoma. Results: Sebaceous carcinoma typically presents as a painless pink or yellow nodule. Diagnosis requires histopathologic examination, and immunohistochemical analysis often assists in the differentiation of sebaceous carcinoma from other benign and malignant skin neoplasms. Sebaceous carcinoma should prompt a workup for Muir-Torre syndrome. Periorbital sebaceous carcinoma has an increased tendency for regional metastasis, although cancer-specific mortality rates are similar among all anatomic locations. Conclusion: Surgery is the preferred treatment for local disease. Limited data suggest that Mohs micrographic surgery may provide superior clinical outcomes, but more research is needed regarding the long-term outcomes. Radiation and systemic chemotherapy are reserved for recurrent or metastatic disease.