Desmoplastic ameloblastoma is an unusual type of ameloblastoma which varies substantially from other variants of ameloblastoma. This case report describes a case of desmoplastic ameloblastoma in a 40-year-old male patient who presented with a swelling in the right maxillary anterior region which was painless in nature. The patient was apprehensive about the asymmetry caused by the massive tumor. Initially, conventional computed tomography was done, and the diagnosis was confirmed by histopathology. Surgical resection of the tumor was done, and a prefabricated prosthetic splint was given postoperatively. The surgical site healed completely, and there was no recurrence at 6-month follow up. The present case report emphasizes the specific histological features of the tumor. Due to its invasive nature, early intervention is needed to reduce the disfigurement, functional hindrance, and other complications caused by the tumor.