We report the case of a 50-year-old woman presenting a primitive adenoid cystic carcinoma of the middle part of skull base diagnosed by transphenoidal biopsy. She was treated by exclusive radiation therapy with cobalt 60 1.25 MeV and the dose received was 66 Gy in 33 fractions. Our patient remains alive 50 months after the completion of treatment. Adenoid cystic carcinoma is a slow growing malignant tumor with perineural invasion which arises more frequently from salivary glands. The occurrence of primary intracranial adenoid cystic carcinoma is exceptional. The best treatment is radical surgery followed by radiotherapy. Chemotherapy is rarely used. Prognosis is worse than for salivary glands.