SummaryBackgroundThe keratocystic odontogenic tumor (KCOT) is a relatively rare, benign neoplasm which develops in the maxilla or mandible, arising from the dental lamina or basal cells of the oral epithelium. It is often found incidentally and brings about late symptoms as it does not cause bone distension for a long time.Case ReportThe presented case is of a young woman with a giant keratocystic odontogenic tumor of the mandible.ConclusionsDespite its rare occurrence, it must be taken into consideration in radiological and clinical diagnostics. Due to the frequent recurrence of KCOT, patients are recommended to be kept under long-term and close radiological supervision.