Adenoid cystic carcinoma (AdCC) comprises of less than 1% of all head and neck cancers and less than 10% of all salivary gland neoplasms. Dedifferentiation/high-grade transformation (HGT) in AdCC is a rare but well known phenomenon which is associated with aggressive clinical behaviour and poor prognosis. We herein report the clinical, cytologic, histologic and immunohistochemical findings of a left submandibular gland AdCC with transformation to high grade carcinomatous and probable dedifferentiation to sarcomatoid component, occurring in a 64 year old male patient. To the author's best knowledge, this is the first case report of such dual transformation occurring in adenoid cystic carcinoma.