An 86-year-old man presented with painless double vision for 6 months. His visual acuity was 20/30 OD and 20/50 OS. He had a palpable mass under the left superolateral orbital rim and bilateral symmetrically decreased tear function. His palpebral fissure was 9 mm OD and margin reflex distance, 5 mm OD, compared with 6 mm and 3 mm, respectively, OS. Exophthalmometry was 13 mm OD and 16 mm OS. He had limited elevation, abduction, and adduction of the left eye with an 8-diopter left hypotropia. Computed tomographic scan of the orbits showed a 2.5 × 1.8-cm, moderately homogenous, hyperattenuating structure in the region of the left lacrimal gland with an area of scalloping in the lacrimal fossa (Figure 1A). The appearance was suspicious for transformation of a pleomorphic adenoma to malignancy. The patient underwent a lateral orbitotomy for resection of the lacrimal mass that was found to be adherent to the periosteum with irregularity of adjacent bone. Because of this, the underlying bone of the lacrimal fossa was sent for histopathological examination. The gland revealed a biphasic tumor containing the typical bilayered pattern of EMC (Figure 1B and Figure 2A, C, and E), with areas of myoepithelial anaplasia (Figure 1C). The latter occupied approximately half of the tumor volume and was composed of a solid component of cells with myoepithelial features and prominent atypia, including nuclear pleomorphism, enlarged nucleoli, mitotic figures, and focal necrosis (Figure 1D). Extension to the anterior, superior, and inferior resection margins was present. The bone specimen was negative for invasion; therefore, the tumor was classified as T2N0M0. Surgical treatment was followed by adjuvant radiotherapy of 6000 cGy because of positive margins. Repeat computed tomographic scan at 8 months postoperatively showed no evidence of recurrence. The patient died 15 months after excision, with no clinical evidence of recurrent disease.