Adenoid cystic carcinomas, the most common malignancies of the lacrimal gland, are rare overall. We describe a patient who presented with right periorbital swelling developing over 5 months and magnetic resonance imaging findings of a soft tissue mass in the lacrimal fossa with invasion of the adjacent bone. The patient underwent right lateral orbitotomy with tumor debulking. Pathologic analysis showed neoplastic cells in a predominantly cribriform pattern, and the patient was diagnosed with an adenoid cystic carcinoma of the lacrimal gland. We review the clinical, radiographic, and histopathologic features of these rare, aggressive malignancies as well as treatment options with reference to the current literature.
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