Desmoplastic ameloblastoma is a rare variant of ameloblastoma. Up until now, less than 150 patients have been reported in the literature. We report a case of desmoplastic ameloblastoma in a 45-year-old female with a painless swelling in the left anterior maxillary region. Fine needle aspiration yielded no fluid. Periapical and panoramic radiographs as well as computer tomography scan showed a mixed lesion with multilocular appearance. The present case deserves special importance be-cause of its unfamiliar appearance, potentially aggressive nature and high chances of misdiagnosis. Moreover, the radio-graphic features of this lesion rarely point towards ameloblastoma. A partial maxillectomy for tumor resection was per-formed and the involved teeth were removed. This report is an attempt to help the dental community in developing familiarity with the clinical presentation and at the same time advocating to develop a high index of suspicion in recognizing such cases.