
Odontogenic keratocyst is an odontogenic developmental cyst. It is derived from remnants of dental lamina. The term was first termed by Philipsen in 1956. OKC was renamed as keratocystic odontogenic tumor (KCOT) in the WHO classifications of head and neck tumors in 2005 due to its aggressive, high recurrence rates and specific histological characterstics and was reclassified into the cystic category in WHO classification of Head and Neck pathology (2017). They are unique odontogenic lesions that have the potential to behave aggressively, that can recur and can be associated with nevoid basal cell carcinoma syndrome. This article focuses on a case of 38 year old female patient diagnosed with odontogenic keratocyst in the left retromolar region and a brief review of literature.