Background. Sebaceous carcinoma of the eyelid is a rare tumor. Treatment can be complicated by noncontiguous spread of the tumor.

Objective. Review a series of patients with sebaceous carcinoma to illustrate clinical presentations, treatments, and outcomes.

Methods. We retrospectively reviewed medical records of patients with sebaceous carcinoma treated at Mayo Clinic (Rochester, MN).

Results. Fourteen patients had sufficient follow-up data available for review. Mean follow-up was 57 months (range 18–134 months). Treatment included wide local excision with frozen and permanent section control (9 patients, 64%), Mohs micrographic surgery (2 patients, 14%), external beam radiation (2 patients, 14%), and exenteration and total parotidectomy with cervical lymph node dissection (1 patient, 7%). Two patients (14%) had local recurrence of the tumor after wide local excision, and 1 patient (7%) had tumor recurrence after Mohs micrographic surgery.

Conclusion. Treatment should be chosen on the basis of the extent of the tumor and the specific needs of the patient. The mainstay of treatment of tumors without orbital involvement has been wide local excision, with the margins checked in both permanent and frozen sections, in combination with conjunctival map biopsies when warranted. Mohs micrographic surgery is an alternative that may provide tissue conservation and lower recurrence rates. Recurrence rates between treatments are difficult to assess because of the small number of cases reported in the literature. In cases with orbital involvement, exenteration may be warranted. Radiation may be useful when surgery cannot be tolerated.