Sebaceous gland carcinoma of the eyelid is a rare slow-growing tumor and is one of the most aggressive malignancies of the eyelid. Diagnosis is often delayed because it can be confused with other periocular lesions. We report the case of a 78-year-old female who presented for the anesthetic aspect of a nodular tumor on the right upper eyelid occurring one year earlier. The patient was treated for three months for recurrent chalazion. Ophthalmologic examination revealed a nodular ulcerated tumor of 1 cm in size adherent to adjacent tissues. Surgical excision was performed with a safety margin of 4 mm. The diagnosis of moderately differentiated sebaceous carcinoma was made by routine morphological methods and immunohistochemical reactions (EMA and Ki-67). Being a rare tumor with considerable morbidity and mortality, early diagnosis and proper treatment are essential for a favorable prognosis and preservation of visual function.

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