Sebaceous carcinoma is a rare malignant tumor of the skin. Although this tumor is not completely understood due to its rarity and the paucity of published reports, it is known to be an aggressive tumor with a high incidence in Asia. Sebaceous carcinomas occur preferentially in the periocular region and require attention not to miss the associated Muir–Torre syndrome. In the case of localized disease, a wide local excision with clear margin followed by adjuvant radiation therapy is usually considered the standard treatment strategy but there is no agreed treatment strategy or standard chemotherapeutic regimen for recurrent metastatic sebaceous carcinoma. We report here two cases of recurrent metastatic sebaceous carcinoma patients who responded to 5-fluorouracil and cisplatin combination chemotherapy, and review the literature. We suggest that 5-fluorouracil-cisplatin can be considered a feasible and effective treatment modality for recurrent sebaceous carcinoma.