Background: Sebaceous carcinoma is an aggressive neoplasm that commonly arises from the meibomian glands of the eyelids and other sebaceous glands of the ocular adnexa. Historic data indicate a nearly 30% local recurrence rate with standard surgical excision. Excision by means of Mohs micrographic surgery may be more efficacious. However, reports documenting the effectiveness of this technique for the treatment of eyelid sebaceous carcinoma have been limited to a few cases. Objective: We report our experience in the treatment of ocular sebaceous carcinoma with the Mohs fresh tissue technique. Methods: Eighteen patients with a diagnosis of sebaceous carcinoma of the eyelid who underwent resection by means of the Mohs fresh tissue technique during the years 1988-1998 were reviewed. Results: Sixteen of the 18 patients were free of disease after an average follow-up of 37 months (11.1% recurrence rate). One patient who experienced local recurrence also had metastatic disease of the parotid lymph nodes (5.6% metastatic rate). The recurrence and metastasis were noted 9 months after excision. The other patient experienced a local recurrence 19 months postoperatively. Both patients exhibited pagetoid spread and involvement of both the upper and lower eyelid at the time of Mohs excision. Conclusion: Mohs surgery offers excellent results when used as the primary treatment modality for sebaceous carcinoma of the eyelid. When compared with historic series of standard surgical excision, Mohs micrographic surgery has a significantly lower recurrence rate and metastatic rate. (J Am Acad Dermatol 2001;44:1004-9.)