Sebaceous carcinomaSebaceous carcinoma (SC) is a complex malignancy that presents significant challenges in diagnosis and treatmentTreatment, primarily affecting the eye and occasionally other body parts. It is a rare form of cancer, constituting about 3% of ocularSebaceous carcinoma,ocular malignancies, with a higher incidence in men and non-Hispanic white individuals aged 60–79. The disease often masquerades as benign eyelidEyelid conditions, making early and accurate diagnosis critical through biopsy. Mohs micrographic surgeryMohs micrographic surgery (MMS) is the gold standard of treatmentTreatment and is effective in managing SC through precise margin control, though the disease’s multifaceted nature necessitates a nuanced approach. The management of SC underscores the importance of comprehensive patient follow-up, given the high rates of local recurrence and the potential for distant metastasis, with guidelines recommending regular monitoring post-diagnosis to adjust treatmentTreatment strategies as needed. This chapter underscores the intricate aspects of diagnosing and treating SC, emphasizing the necessity for vigilance in recognizing new lesions and the critical role of long-term patient follow-up in managing this challenging malignancy.