Extraosseous ameloblastoma (EA) is a rare odontogenic tumor which comprises about 1% of all ameloblastomas. Extraosseous location is the peculiar feature of this type of tumor, which is otherwise similar to the classical ameloblastoma. The exact etiology of EA is not yet known. The clinical appearance may vary; it presents as a slow-growing, firm, painless mass with a sessile or pedunculated base with a smooth surface. The radiographic findings are nonspecific; diagnosis is based on histopathological features. It poses a diagnostic and therapeutic challenge due to its morphology and biological behavior. This article aims to elucidate rare case of EA of maxillary gingiva in a 40-year-old male patient and also an attempt has been made to discuss its pathogenesis in light of current information from the literature.
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