Ameloblastoma is benign, locally aggressive, polymorphic neoplasm of proliferating odontogenic epithelial origin. Desmoplastic variant account for 4-5% of all ameloblastomas. The present study describes the case of a patient that experienced numerous recurrences in the maxilla and also discusses recommendations for treatment. A 18 year old female underwent left maxillectomy for complain of swelling over left cheek. Histopathology report revealed it to be a desmoplastic ameloblastoma. Patient than received external beam radiotherapy to head and neck region 60Gy in 30 fractions and then the patient had recurrence four years later with huge mass seen on left side of hard palate with extension from soft palate. Patient received chemotherapy and then planned for radiotherapy 20 Gy in 5 fractions after which wide local excision was done. Now patient is asymptomatic and on follow up.
