The extraosseous odontogenic tumors may raise diagnostic challenges due to their imitation for reactive gingival lesions. Among them, the peripheral dentinogenic ghost cell tumor (PDGCT) is an extremely rare benign odontogenic neoplasm with up to 30 cases published in the literature. PDGCT occurs in older adults most commonly in the anterior mandibular region. We present a new case of PDGCT located in the maxillary premolar gingiva of a 67-year old man along with differential diagnosis. The lesion was presented as a broad based tumor covered by ulcerated mucosa. The histopathologic examination revealed the typical features of PDGCT exhibiting multiple irregular masses of ghost cell aggregates, dysplastic dentin in close relation to ameloblastoma-like epithelial islands. Despite of the rarity of this entity, PDGCT should be included in the differential diagnosis of the gingival tumors.
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