Adenoid Cystic Carcinoma is a very rare cancer typically originating in the salivary glands. Its course is slow but persistent with most patients surviving 5 years, with a very poor prognosis after 10 years due to recurrent tumors and metastasis to the lungs, liver, and bone. Adenoid cystic carcinoma occurs slightly more frequently in women than in men. Treatment is surgical ressection whenever possible. There is no current effective chemotherapy for metastatic or unresectable tumors caused by this disease.