This article describes the clinical, histological, radiological characteristics, and treatment of unicystic ameloblastoma by presenting a clinical case of an 11-year-old patient, over a period of 2 years of clinical control and follow-up, in which histopathological diagnosis and radiographic control play a crucial role in diagnosis and the choice of an appropriate treatment plan. In this case, the patient underwent marsupialization, and after 4 weeks, enucleation in the mandibular symphysis and parasymphysis, along with odontectomy under general anesthesia. A treatment success rate of 100% without recurrence was achieved, and postoperative follow-up every 6 months was recommended.