AbstractCutaneous sebaceous carcinoma occurs almost exclusively on the head and neck and has a significant propensity for recurrence and metastasis. It is easily mistaken for benign conditions, resulting in inappropriate management. Thus, it is important to maintain a high index of suspicion. Despite previous reports, sebaceous carcinoma may occur with similar frequency in Asians and whites. Recent genetic data suggest there are multiple mutational groups of sebaceous carcinoma, paving the way for targeted treatment. After a diagnosis, investigations for staging and for Muir–Torre syndrome should be considered. The available evidence on the treatment options for sebaceous carcinoma is discussed, and specific recommendations for management are made.