Calcifying epithelial odontogenic tumor (CEOT) is a rare neoplasm, which accounts for < 1% of all odontogenic tumors. CEOT occurs more frequently in adults with a peak incidence in the 5th decade of life and is extremely rare in the pediatric population. We present a case of a 13-year-old girl who was found to have a mandibular CEOT. We summarize the radiological features, pathological findings, clinical management and literature review focusing on this entity in children.Electronic supplementary materialThe online version of this article (10.1007/s12105-019-01009-1) contains supplementary material, which is available to authorized users.