International Journal of Research and Reports in Dentistry

Orthokeratinized odontogenic cyst (OOC) is a rare developmental odontogenic cyst of the jaws with an uncertain etiology. Formerly considered a variant of the odontogenic keratocyst, OOC is now recognized as a distinct pathological entity because of its characteristic histopathological features and comparatively less aggressive biological behavior. It most commonly occurs in the posterior mandible and demonstrates a male predilection. Clinically, OOC is usually asymptomatic, although pain and swelling may occur secondary to infection. Radiographically, it commonly presents as a well-defined unilocular radiolucency, often associated with impacted teeth, and may resemble a dentigerous cyst despite differences in pathogenesis and clinical behavior. We report a rare case of OOC in a 40-year-old female who presented with swelling in the left vestibular region. Radiographic examination revealed a multilocular radiolucency. Histopathological analysis demonstrated orthokeratinized epithelium with focal areas of non-keratinization in inflamed regions, along with the presence of multinucleated giant cells. This case emphasizes the importance of careful clinical, radiographic, and histopathological evaluation for accurate diagnosis, appropriate management, and favorable prognosis.