Acinic cell carcinoma is a rare neoplasm nearly always observed in the salivary glands. Our experience documents a case of this pathology, occurring in the lacrimal gland, never reported in the literature before. A 59-year-old white woman was admitted with reduced vision and a painless swelling in the area of the right lacrimal gland. Computerized axial tomography showed an intraorbital radio-opaque mass compressing the eyeball from behind. The patient underwent right temporal orbitotomy and total removal of the lesion. The surgical specimen was constituted by a cystic mass containing citrine liquid. Microscopic examination proved the lesion to be an acinic cell carcinoma. Differential diagnostic problems and similarity to salivary gland acinic tumors are discussed.