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Abstract:  Sebaceous carcinoma is an aggressive, adnexal, rare malignant tumor that may arise in ocular or extra-ocular sites. Extraorbital sebaceous carcinoma is exceptional in childhood. We report a 12-year-old boy with an ocular sebaceous carcinoma who was first seen with an asymptomatic firm, cutaneous nodule on the right eyebrow. The tumor developed slowly within 1 year. Histologically, it was an undifferentiated sebaceous carcinoma. The patient had surgery with wide surgical margins. He was alive and free from disease at a follow-up of 34 months. Close follow-up of this tumor is recommended because of the risk of aggressive behavior.