Odontogenic keratocysts are jaw developing cysts that need to be properly diagnosed because they can grow aggressively locally, recur, and have a genetic relationship. One of the most contentious changes in the terminology of odontogenic lesions in recent years has been the renaming of odontogenic keratocysts as keratocystic odontogenic tumors in 2005, followed by the WHO's designation of an odontogenic cystic lesion in 2017 after a 12-year period. Odontogenic keratocysts are jaw developing cysts that need to be properly diagnosed because they can grow aggressively locally, recur, and have a genetic relationship. In 2005, odontogenic keratocysts were renamed as keratocystic odontogenic tumors. Twelve years later, the World Health Organization classified these lesions as odontogenic cystic lesions in 2017, has been one of the more contentious alterations to the nomenclature of odontogenic lesions in the last few years. In order to explain the significance of this lesion, this article examines the etiopathogenesis, clinical, radiographic, histological, immunohistochemical, and therapeutic aspects.