Abstract
The orthokeratinized odontogenic cyst (OOC) is a rare developmental odontogenic cyst that has been considered as a variant of the keratocystic odontogenic tumor until Wright (1981) defined it as a different entity. Recognition of OOC as a unique entity has long been due, yet its inexplicable clinical, radiographic presentation resembling dentigerous cyst due to its association to an impacted tooth, and its histological features makes it rather perplexing. This is the report of a case of OOC in relation to an impacted maxillary canine and its immunohistochemical analysis with Ki-67.