Purpose: To report the frequency and clinical features of internal visceral ma lignancy in an unselected series of patients with sebaceous gland carcinoma (SGC ).Methods: A non-comparative retrospective case series of consecutive patients with a histologically proven diagnosis of periocular sebaceous cell carcinoma tr eated at the University Eye Hospital, University of Erlangen-Nürnberg between 1981 and 2000. Results: Twenty three patients were identified, each with one tum or. Men and women were almost equally affected with a mean age at diagnosis of 6 2 years (range 37-83 years). The majority of tumors were located in the upper l id, but five tumors were situated in the lower lid or caruncle. In 22 of the 23 patients the diagnosis at referral was either incorrect or not suspected. In all cases, the SGC was treated by surgical excision. In the systems review, 11 of t he 23 patients (48%) had evidence of previous systemic malignancy, the most com mon being colorectal cancer followed by liver and bronchial carcinoma. Forty-fi ve percent of patients with SGC and internal visceral malignancy eventually died as a result of their tumors. Conclusion: Periocular SGC remains an underdiagnos ed entity for which a high level of clinical suspicion is critical for early rec ognition. Almost half the patients additionally had an internal visceral maligna ncy. Internal visceral malignancy in unselected patients with SGC may be more co mmon and have a more guarded prognosis than has been appreciated. Purpose: To report the frequency and clinical features of internal visceral ma lignancy in an unselected series of patients with sebaceous gland carcinoma (SGC). Methods: A non-comparative retrospective case series of consecutive patients with a histologically proven diagnosis of periocular sebaceous cell carcinoma tr eated at the University Eye Hospital, University of Erlangen-Nürnberg between 1981 and 2000. Results: Twenty three patients were identified, each with one tum or. Men and women were almost equally affected with a mean age at diagnosis of 6 2 years ( range 37-83 years). The majority of tumors were located in the upper l id, but five tumors were situated in the lower lid or caruncle. In 22 of the 23 patients the diagnosis at referral was either incorrect or not suspected. In the systems review, 11 of 23 patients (48%) had evidence of previous systemic malignancy, the most com mon being colorectal cancer followed b y liver and bronchial carcinoma. Forty-fi ve percent of patients with SGC and internal visceral malignancy terminated died as a result of their tumors. Conclusion: Periocular SGC remains an underdiagnos ed entity for which a high level of clinical suspicion is critical for early rec ognition. Almost half the patient additionally had an internal visceral maligna ncy. Internal visceral malignancy in unselected patients with SGC may be more co mmon and have a more guarded prognosis than has been appreciated.