Adenoid Cystic Carcinoma (ACC) is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region [
1]. It is a slowly growing but highly invasive cancer with high recurrence rate. Lymphatic spread to local lymph nodes is rare. Hematogenous spread, however, occurs often in the course of the disease [
2]. Intracranial ACC even is more rare and has been reported as 4 – 22% of ACC [
3]. It could be primary or secondary which could occur either by direct invasion like in our case, hematogenous spread, or perineural spread [
4,
5]. Perineural spread of ACC has long been recognized. The literature revealed that the region of Gasserian ganglion to be the most common site of involvement (35.8%) [
2,
3,
6,
7], while cavernous sinus was involved in 15.1% [
3‐
5,
8,
9]. Presenting signs and symptoms are related to the anatomical site of the lesion. Facial pain, parasthesia in trigeminal distribution is commonly reported reflecting the frequency of involvement of gasserian ganglia, and possibility of perineural spread along the trigeminal nerve. Involvement of cavernous sinus could be asymptomatic [
3] or could present with involvement of either 3
rd, 4
th, 5
th, 6
th and internal carotid artery [
8,
9]. Literature is consistent that the time between onset of neurological signs and symptoms, and the time of diagnosis range between few months to 3 years [
3,
6,
7,
10]. However, one study suggests that the duration could be several years [
8].