Salivary gland tumours constitute 3% of all head and neck neoplasms. Pleomorphic adenomas arising in the maxilla are extremely rare, with only few cases reported to date. Radiographically and clinically, these lesions have the appearance of osteolytic processes or odontogenic cysts and the diagnosis represents a challenge. The surgical treatment of intraosseous glandular neoplasms is debated because of the few cases reported in literature. The Authors describe a rare case of intraosseous pleomorphic adenoma occurring in the maxilla along with a complete review of literature.