Adenoid cystic carcinoma is a rare tumor arising from the minor salivary glands;, the palate being the commonest site. Distant metastasis and perineural invasion areis common in adenoid cystic carcinoma. Diagnosis of adenoid cystic carcinoma is made usually with the help of clinical features, radiographic features and histologic features. We reported a case of adenoid cystic carcinoma of palate involving left maxillary sinus. The diagnosis of the case and brief review of literature of adenoid cystic carcinoma is discussed. The aim here is to highlight the importance of diagnosis, treatment and long-term follow-up of the patients with adenoid cystic carcinoma.