Desmoplastic ameloblastoma (DA) accounts for 4–13% of all ameloblastomas and presents unique characteristics, such as the ability to appear as a mixed radiolucent–radiopaque image radiographically, differing from the typically radiolucent appearance of CA. The biological behavior of DA is like that of other CA variants and therefore should be treated similarly. The present study, reported a case of DA affecting a young female, highlighting its clinical, radiographic and treatment outcomes. Intraorally, a firm submucosal enlargement was observed in the anterior mandibular area. Radiographically, a radiolucency was observed between the the lower left lateral incisor and canine teeth. An incisional biopsy was performed, confirming the histopathological diagnosis of DA. The patient was submited to radical surgery under general anesthesia and there are no signs of the tumor after a 2-year follow-up. Therefore, the present case demonstrated the importance of early diagnosis and appropriate radical surgical management, which allowed effective tumor control with preservation of mandibular integrity and satisfactory functional outcome, with no recurrence after two years.