Aim: To report a rare presentation of adenoid cystic carcinoma (ACC) of the lacrimal gland. Method: This is a case report presenting clinical, radiographic, and histopathologic findings. Results: A 72-year-old female with no reported ocular symptoms was found to have an incidental right orbital mass on imaging. Additional studies revealed multiple liver lesions, which were biopsied and found to be consistent with ACC. She was then referred to the ophthalmology service, where ocular examination demonstrated 2.5 mm of right proptosis with elevation and abduction deficits. Diplopia could be elicited in extreme upgaze and right lateral gaze. An excisional biopsy of the orbital mass was performed, with histopathology confirming the diagnosis of primary ACC of the lacrimal gland, thereby also supporting the initial suspicion that the hepatic ACC lesions represented metastases. Conclusion: The authors describe a rare presentation of ACC of the lacrimal gland, initially asymptomatic, with metastatic lesions restricted to the liver at the time of diagnosis. Three previous cases of ACC with isolated metastatic hepatic lesions at the time of diagnosis have been reported; all of these cases localized the primary tumor to the salivary glands.