Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare clinicopathologic entity that has been the subject of considerable confusion and debate. ALHE shares both clinical and histopathologic features with Kimura disease (KD). Because of this overlap, ALHE and KD have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing ALHE and KD as two separate nosological entities, based on their clinical, laboratory, and especially histopathologic findings. ALHE commonly affects women in the third and fourth decades, with head and neck involvement. ALHE has been described rarely in the orbit, eyelids, and lacrimal gland. We report a case of ALHE involving the left lacrimal gland in a 40-year-old Mexican woman. Because ALHE can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, it should be considered in the differential diagnosis of orbital lesions occurring in adults.