The adenoid cystic carcinoma (ACC) is an uncommon slowly growing malignant salivary gland tumor. Due to the distribution of minor salivary gland, ACC is rarely occurred in the upper anterior alveolar ridge. In this paper, the author presents a case of adenoid cystic carcinoma is original from upper anterior alveolar ridge and extended to palate, upper labial mucosa and adjacent maxillary bone, with invasion into nasal cavity. The further treatment after surgery, and the related prognosis will be discussed throughout literature.