Primary adenocarcinoma of the lacrimal gland with sebaceous differentiation is an extremely rare tumor. Only 11 cases have been reported in the literature, none in children. A 4-year-old girl presented with a rapidly progressing mass in the left lacrimal gland fossa. An incisional biopsy followed by partial orbital exenteration confirmed the diagnosis of adenocarcinoma with sebaceous cell differentiation. Currently, no previous reports of this malignancy in childhood have been published. This case underscores the necessity of expanding the presenting age that adenocarcinomas with sebaceous differentiation of the lacrimal gland can occur.
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