Abstract:The keratocystic odontogenic tumor is a benign intraosseous neoplasm derived from remnants of the dental lamina and it occurs with high frequency. Regarding histological characteristics, it has a high recurrence rate which is one of the main therapeutic problems. Also, it presents high local aggressiveness, expressed in cortical expansion, delayed eruption and displacement of teeth, blood vessels and nerves. At present, there are various treatments, being ideal the one which presents the lowest risk of recurrence with low morbidity for the patient. In this review, the main histopathological, clinical and therapeutic aspects of this oral pathology are discussed.