Abstract
An orthokeratinized odontogenic cyst (OOC) is a rare developmental odontogenic cyst characterized by orthokeratinized stratified squamous epithelium. It was previously considered a variant of an odontogenic keratocyst (OKC) but has since been classified as a separate entity due to differing biological behavior. We present a case of an OOC in a 17-year-old female patient with extra-oral swelling on her right ramus region. Radiolucency in both the jaws was discovered incidentally on routine OPG examination. These lesions were surgically enucleated, and histopathological examination confirmed the diagnosis. This report highlights the clinical, radiographic, and histological characteristics of OOCs with their rarity of multiple incidence and reviews pertinent literature.