A 41-year-old woman presented with several days of right eye pain and blurred vision. Examination and radiologic workup were consistent with a veno-lymphatic malformation, including demonstration of cystic fluid–fluid levels on imaging. Histopathology supported the presence of cystic spaces but revealed the final diagnosis of lacrimal epithelial–myoepithelial carcinoma, a rare neoplasm typically associated with the salivary gland. This represents the first reported case of lacrimal epithelial–myoepithelial carcinoma presenting with pain and evidence of intralesional cysts on radiographic and histopathologic evaluation. Better characterization will enable recognition, complete surgical excision, and potential adjuvant radiation therapy.
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