We report a case of adenocystic carcinoma (cylindroma) of the maxillary sinus with intracranial extension in a 35-year-old man with no previous clinical history. The patient underwent primary neurological and maxillofacial surgery. He then developed two successive recurrences of the tumor in the temporo-parietal area of the brain treated by surgery and radiotherapy for the first one and surgery alone for the second. The patient finally died 4 years and 2 months after the diagnosis. Adenoid cystic carcinomas are malignant epithelial neoplasms that arise mainly from salivary glands (14% of the salivary tumors), and less frequently from other structures. Their histological pattern can be either tubular, cribriform or solid, with different prognosis. They invade local areas (perineural spaces) and also metastasize to the lung, liver, bone, regional lymph nodes. Treatment mainly consists in combined surgery and radiation therapy. Chemotherapy might be also used.