Expect the unexpected – A rare case of Orthokeratinized Odontogenic Cyst and its surgical management

Orthokeratinized Odontogenic Cyst (OOC) is a rare, developmental odontogenic cyst of the dental lamina. It was initially defined as the uncommon orthokeratinized variant of the Odontogenic Keratocyst (OKC), until the World Health Organization’s (WHO’s) classification in 2005 and 2017, where it was separated from the Keratocystic Odontogenic Tumor (KCOT) and has been included as a separate entity from the category of developmental odontogenic cysts respectively. It presents as a unilocular radiolucent lesion involving the posterior mandible and is frequently related to impacted teeth, often similar to other odontogenic cysts. Due to low local aggressiveness and less proliferative activity, it has to be differentiated from the other cysts in terms of surgical management. Here we report a rare case of OOC involving the maxilla along with an impacted canine and discuss the surgical management and why a secondary surgical intervention is unnecessary.