The calcifying odontogenic cyst (COC) is an uncommon developmental odontogenic cyst, the oral counterpart of Malherbe's cutaneous calcifying epithelioma (pilomatricoma). This article presents two unique cases of calcifying odontogenic cysts each exhibiting distinctive histopathological features and its literature review. One case with an unexpected finding of cholesterol granuloma (CG), a rare occurrence in non-inflammatory cysts within an unusual location between two maxillary central incisors. One more instance involves the presence of a compound odontome in conjunction with COC. The cases underscore the clinical and histopathological diversity of COC and highlight the importance of radiological and histopathological assessments for accurate diagnosis. The unexpected association of COC with cholesterol granuloma challenges traditional diagnostic expectations. Additionally, the second case suggests that COCs may warrant sub-categorization to better understand their varied presentations and biological behavior. This article contributes to the expanding knowledge of COC, emphasizing the significance of documenting rare cases to enhance comprehension of its nature, pathogenesis, and oral cavity origin.
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