The diagnosis and treatment of maxillofacial cystic lesions requires careful evaluation and correlation of the clinical presentation and radiological studies. The Pindborg tumor, also knownas the calcifying epithelial odontogenic tumor, is a locally invasive benign neoplasm, with onlyaround 300 cases being published to date. This study presents a new case of this already uncommonneoplasm, not associated with an impacted tooth, and describes the clinicopathological features ofthis rare entity, along with a review of other reported cases. Despite surgery having been recognizedas the treatment of choice for the Pindborg tumor, no firm consensus exists concerning the extensionof surgical resection.