Background:Peripheral Calcifying Epithelial Odontogenic Tumors (CEOT) rich in clear cells are a rare entity in the oral cavity, with only 14 previous case reports in the English literature. None have discussed recommended treatment approaches for extraosseous CEOT.Objective:This brief descriptive review describes a treatment approach for peripheral CEOT including the clear cell variant.Study design:A complete review of all well-documented extraosseous case reports with an emphasis on the treatment was performed. Additionally, the present article reports a case of a 21-year-old woman with an asymptomatic swelling in the gingiva finally diagnosed as peripheral CEOT abundant in clear cells.Results:Twenty-four cases of peripheral CEOT were described; conservative surgery was the first treatment approach in approximately 80% of cases, with only one recurrence.Discussion:Clear cell finding was not associated with more aggressive behavior.Conclusion:Conservative surgery may be an advantageous approach for this group of peripheral lesions with or without clear cells, with a recurrence rate of approximately 4%.