Adenoid cystic carcinoma (ACC) is an epithelial tumor that occurs primarily in the major salivary and lacrimal glands. The skin can be involved secondarily through direct or perineural invasion, or as a site of distant metastasis. Primary cutaneous adenoid cystic carcinoma is a rare form mostly seen on the scalp and chest, but has been reported in the eyelids. Eyelid ACC can arise from accessory glands in the conjunctiva and cutaneous accessory lacrimal glands of Krauss and Wolfring. It is locally aggressive with a tendency for perineural invasion. Treatment is with wide local excision. In the orbit ACC is the most common primary epithelial tumor of the lacrimal gland. Patients usually present with pain, ptosis, diplopia, proptosis and a downward medial displacement of the globe. Treatment is with en bloc excision including adjacent bone and adjunctive radiotherapy for advanced lesions.