Orthokeratinized odontogenic cyst (OOC) is an odontogenic cyst was initially termed as the uncommon orthokeratinized type of odontogenic keratocyst by the World Health Organization. It usually occurs in mandible. Various studies have shown that OOC has typical characteristic clinicopathologic features when compared to other developmental odontogenic lesions such as dentigerous cyst and the keratocystic odontogenic tumor (KCOT). Rare recurrence was noted after surgical removal of the lesion. The purpose of this article is to present a case of OOC arising in the posterior mandible and emphasize on differentiating it from the KCOT and dentigerous cyst.