<i>Background:</i> We analyzed the clinicopathological features and prognosis in patients with periocular sebaceous gland carcinoma. <i>Methods:</i> Retrospectively, we studied the records of 24 cases of sebaceous gland carcinoma treated in our clinic between 1999 and 2004. The reviewed data included demographic information, initial clinical and histopathological diagnosis, anatomic tumor location, details of treatment, tumor involvement of resection margin, and outcomes such as local recurrence, distant metastasis and survival. <i>Results:</i> Local recurrence developed in 6 cases (25%) at a median of 20 months from initial diagnosis. Only 2 patients (8.3%) died of systemic metastasis to brain and bone from primary lacrimal gland carcinoma. The local recurrence rate in the complete excision group (5.9%) was significantly lower compared with the incomplete excision with irradiation group (71.4%; Fisher’s exact test, p = 0.003). We observed a shorter duration of symptom, a higher rate of eventual exenteration and local recurrence, and a shorter time interval to local recurrence from initial management in poorly differentiated tumors compared with moderately differentiated tumors. <i>Conclusion:</i> It is critical to achieve a negative tumor margin in both frozen and permanent section to prevent local recurrence. Early aggressive management is required in sebaceous gland carcinoma of the lacrimal gland, as its mortality is much higher than that of eyelid origin.