Orthokeratinizedodontogenic cyst (OOC) is a relatively rare developmental odontogenic cyst that occurs in the jaw bones. It was initially defined by Wright in 1981 as Orthokeratinized variant of OdontogenicKeratocyst (OKC) with distinct clinicopathological features. However, after undergoing a few revisions by World Health Organization (WHO), OOC was finally considered its own entity as another type of odontogenic cyst in 2010. Orthokeratinizedodontogenic cyst (OOC) is a distinct entity from OdontogenicKeratocyst (OKC), and should not be referred as orthokeratinized variant of OKC .These cysts are often asymptomatic until becoming secondarily infected.It also can grow large in size causing cortical expansion and presents as a swelling, along with pain, although in the majority of cases, it can be incidentally detected during radiographic examination. Based on computerized tomography scan (CT) findings, the OOC may appear as a multilocular radiolucencyat the posterior mandible that may be suggestive of ameloblastoma or OKC and can be mistakenly diagnosed clinically as a Dentigerous Cyst due to involvement of an impacted tooth. Hence, incisional biopsy of the cyst is required prior to definitive treatment. Histopathologically, OCC shows a cystic cavity lined by thin (5-8 cell layers thick) and uniform stratified squamous epithelium with prominent granular cell layer and orthokeratin. Due to less aggressive behaviour and the recurrence rate of OOC is low, hence surgical enucleationand curettage of the cystic lesion with the removal of involved teeth is the mainstay of the treatment. Despite of low recurrence rate, regular follow up is still recommended.The purpose of this article is to present a rare case of OOC arising in the right angle of mandible of a male patient which is associated with an impacted lower right wisdom tooth.

Keywords: Orthokeratinizedod ontogenic cyst, Odontogenic cyst, Odontogenickeratocyst, Wisdom tooth, Enucleation