Calcifying epithelial odontogenic tumors are rare odontogenic neoplasms that account for approximately 1% of all odontogenic tumors. The extraosseous variant is extremely rare, with only few cases being described in the literature. To our knowledge, only 22 cases have been reported over a period of 44 years. Therefore, the aim of this study was to present a critical literature review comprising the period from 1966 to 2010. In addition, we discuss the clinical presentation, diagnosis, and subsequent treatment of a patient with a gingival calcifying epithelial odontogenic tumor in the anterior mandible. This study shows that even though being an extremely rare neoplasm the gingival calcifying epithelial odontogenic tumor should be included in the differential diagnosis by general dentists of gingival lesions due to its clinical similarity to other oral hyperplastic or reactive lesions. A conservative surgical approach can be adopted in view of the nonaggressive behavior of this tumor. Recurrence is low and the prognosis is excellent