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Peripheral calcifying epithelial odontogenic tumor (CEOT) is a rare benign epithelial odontogenic neoplasm,
accounting for only about 1% of all odontogenic tumors, with the extraosseous variant representing a small fraction
of cases. Despite its rarity, this lesion can mimic various reactive and neoplastic gingival conditions, making clinical
recognition challenging. The clear cell variant of peripheral CEOT is even rarer and may mimic malignant tumors in
histopathology, underscoring the need for thorough clinicopathological and radiographic assessment with
histopathological exclusion. Because it frequently presents as an innocuous, slow-growing gingival mass, it may be
clinically misdiagnosed as hyperplasia, pyogenic granuloma, hamartoma, or salivary gland pathology. Multiple
biopsies may be required for large or heterogeneous lesions. A total of 50 cases have been documented over the past
five decades (1966-2025), underscoring the extreme rarity of this condition. This review synthesizes reported cases to
reinforce the importance of considering peripheral CEOT in the differential diagnosis of localized gingival
enlargements. Conservative surgical excision remains the preferred treatment, with low recurrence rates and an
excellent overall prognosis.