Abstract
Adenoid cystic carcinoma (ACC) is a rare slow-growing but aggressive malignant tumor arising from the epithelial cells of mucous-secreting glands. Primary intracranial ACC is one of the rarest entity. We report a case of a 61 years old male presenting with difficulty in swallowing, slurring of speech, generalized body weakness. Patient had residual right cerebellopontine angle (CPA) mass causing midline shift and fourth ventricular obstruction on MRI. Patient underwent right retrosigmoid craniotomy with excision of CPA mass. Histopathological examination confirmed the case as primary intracranial ACC.