The odontogenic keratocyst (OKC), which makes up approximately 10% of odontogenic cysts, is a locally-aggressive cystic lesion that affects the maxilla or the mandible and is capable of causing major destruction. OKCs have been a topic of debate ever since they were discovered and named. The origin of OKC is still debatable, the OKC mostly occur intraosseusly and is thought to be derived from odontogenic epithelium as the dental lamina and its remnants after the organ has served its purpose. The WHO had classified OKC under ‘developmental odontogenic cysts of jaw' in the 1971 and 1992 classifications . Nevertheless, the 2005 WHO classification controversially considered OKC an odontogenic neoplasm and gave it the name ‘keratocystic odontogenic tumor' (KCOT) . The reasons for this change were the lesion's high recurrence rate, aggressive clinical behavior, association with nevoid basal cell carcinoma syndrome and mutations in the PTCH tumor suppressor gene. The designation of OKC changed once again in the 2017 WHO classification, reverting back to the more accepted term ‘odontogenic keratocyst'. OKCs grow in an antero-posterior direction, infiltrating the cancellous bone of the jaws, often without obvious bone expansion, so it is commonly asymptomatic despite its aggressive nature. The presence of multiple OKCs is especially correlated to nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin-Goltz syndrome , the radiographic appearance of OKC ranges from well-defined unilocular lesions to extensive multilocular lesions with ill-defined borders. This review will cover the history of this lesion's classification , nomenclature, as well as its histogenesis, clinical presentation, histopathology, radiography, genetics, treatment, prognosis and complications.