Adenoid cystic carcinoma (ACC) is a relatively common malignant neoplasm wh
ich occurs in the head and neck region, particularly in the salivary glands
. It is a slow growing, locally aggressive neoplasm with a unique feature o
f 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 g
ood response to treatment with surgery followed by post-operative radiother
apy and has remained disease free, 32 months after the completion of treatm
ent.