PURPOSE: To report a patient with an adenocarcinoma in a pleomorphic adenoma of the lacrimal gland. Adenocarcinoma constitutes a distinct group of epithelial malignancies of the lacrimal gland. METHODS: The clinical presentation, workup, surgical approach, and pathological findings were reviewed. RESULTS: A 56-year-old man presented with a 3-month history of a lacrimal fossa mass. This patient presented a painless mass in the upper outer eyelid with significant displacement of the globe. The tumor was localized to the lacrimal gland. Radiological investigations showed a round, well-defined lesion in the fossa of the lacrimal gland. We performed a lateral orbitotomy; en bloc resection was accomplished. The final specimen showed an adenocarcinoma in a pleomorphic adenoma. No recurrences were detected during follow-up. CONCLUSION: Although adenocarcinoma has rarely been reported in association with a pleomorphic adenoma of the lacrimal gland, this combination can exist. If the malignant tumor is limited in the pleomorphic adenoma, the prognosis appears to be better than in cases of local extension. Adjuvant therapy, radiation or others, are not useful.