Ameloblastoma is one of the most common odontogenic tumors. Desmoplastic Ameloblastoma(DA) is a relatively rare variant. Compared with conventional ameloblastoma, DA shows distinct radiographic features which mimic a fibro-osseous lesion i.e with mixed radiopacity and radiolucency. The local invasive nature and high recurrence rate of this variant necessitates surgical treatment with resection. DA most commonly affects anterior region of the jaws. We present here a unique case of aggressive, giant desmoplastic ameloblastoma in a 50 years old female. Keywords: Aggressive, Desmoplastic ameloblastoma, Odontogenic tumor, Stromal Desmoplasia Received: 22 February, 2019 Revised: 28 February, 2019 Accepted: 5 March, 2019 Corresponding author: Dr. Anand Nikunj, PG student, Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, India This article may be cited as: Nikunj A, Meshram D, Deshpande M, Jadhav D, Rajurkar SG. Aggressive giant desmoplastic ameloblastoma -A unique case. Int J Res Health Allied Sci 2019; 5(1):92-93.