<![CDATA[Background: Ameloblastoma is a benign epithelial odontogenic tumor with a high recurrence tendency, predominantly found in the mandible. Follicular ameloblastoma is the most common histopathological subtype of the ameloblastoma. Its complex histopathology can complicate diagnosis, particularly when limited biopsy sampling fails to represent the entire tumor. Objectives: This case report aims to highlight the importance of comprehensive histopathological evaluation of the resected specimen in establishing a definitive diagnosis of ameloblastoma. Case: A 35-year-old female presented with a painless, progressively enlarging right mandibular mass. Incisional biopsy indicated ossifying fibroma, while CT scan revealed a destructive, expansile lesion measuring 3.1x 3.3 x 5.23 cm inconsistent with a fibro-osseous lesion. Specimen review subsequently suggested mixed-type ameloblastoma. This case highlights the importance of comprehensive histopathological evaluation of the complete resected specimen, as limited biopsy sampling may not capture the predominant architectural pattern in histologically heterogeneous tumors such as ameloblastoma. Segmental resection with iliac crest bone graft reconstruction was selected to ensure adequate tumor removal and restore mandibular continuity. Managing Cases: Given this diagnostic complexity, segmental mandibulectomy with immediate iliac crest bone graft reconstruction and plate fixation was performed. Histopathological examination of the definitive resected specimen ultimately confirmed follicular-type ameloblastoma, with no evidence of malignancy. Conclusion: This case demonstrates that histopathological heterogeneity of ameloblastoma may lead to diagnostic discordance. Segmental mandibulectomy with immediate reconstruction served as both the definitive diagnostic and therapeutic solution for extensive mandibular involvement.]]>
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