Orthokeratinized odontogenic cyst (OOC) is a rare developmental odontogenic cyst occurring in the jaw with debated etiology. It was originally believed to be a variant of odontogenic keratocyst (OKC) but is now considered to be a distinct entity. The majority of the cases occur in the third and fourth decades of life. The common site is the mandibular posterior region with a male predilection. Swelling is the most common symptom which may be accompanied by pain, although in most cases, the lesion is asymptomatic. These lesions mostly present as unilocular radiolucency often associated with an impacted tooth. They may mimic dentigerous cyst and OKC in radiologic and histopathologic presentation, however, differ in biological behavior, pathogenesis and prognosis in comparison. Hence, making an accurate diagnosis is essential. This article describes an incidental finding of OOC in a 28-year-old female during radiographic investigation for orthodontic treatment. This case showed some rare features such as multilocular radiolucency, nonkeratinized epithelium in areas of inflammation, few cholesterol clefts with giant cells, presence of dentinoid-like material and dystrophic calcification in the capsule.
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