Keratocystic odontogenic tumor (KCOT) comprises a unique pathological entity characterized by aggressive or destructive behavior and propensity to recurrence. Usage of the term odontogenic keratocyst (OKC) had been under a lot of dispute from the time it was introduced in 1956. The World Health Organization (WHO) reclassified this lesion in 2005 as a KCOT and defined it as “a benign uni or multicystic intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and potentially aggressive, infiltrative behavior.” It may be solitary or multiple. Long standing KCOTs have been reported to transform into primary intraosseous carcinoma (PIOSCC) or an ameloblastoma.