Among primary lacrimal gland tumors, adenoid cystic carcinoma (ACC) is the most common malignant epithelial neoplasm; it is characterized by local intracranial invasion. A case with unusual dumbbell-type intracranial extension representing cavernous sinus syndrome is described. A 49-year-old woman was admitted to our hospital with right cavernous sinus syndrome. Computerized tomographic (CT) scans and magnetic resonance (MR) imaging demonstrated well-enhanced intraorbital and middle fossa tumors mimicking multifocal mass lesions. Operative findings revealed an ACC originating from the lacrimal gland and extending into the right cavernous sinus and middle fossa along the nerve sheath in the superior orbital fissure. Although MR image findings of intracranial ACC often resemble the image findings for meningiomas, intracranial ACC is very aggressive in comparison with meningioma. It is best treated surgically and aggressively.