ABSTRACT
Adenoid cystic carcinoma (ACC) is a relatively common malignant neoplasm which occurs in the head and neck region, particularly in the salivary glands. It is a slow growing, locally aggressive neoplasm with a unique feature of perineural spread. Intracranial extension can occur by direct extension, hematogenous metastases or by perineural spread. However, the occurrence of primary intracranial ACC with no evidence of a primary, is rare, with only ten cases reported in reviewed English literature until now. One such case is presented here with brief review of literature. This patient showed a good response to treatment with surgery followed by post-operative radiotherapy and has remained disease free, 32 months after the completion of treatment.
