Report of two cases of syndromic and non-syndromic multiple keratocysts

Introduction: Keratocystic odontogenic tumor (KCOT) is a benign odontogenic lesion with aggressive behavior and high recurrent rate. In some rare cases, multiple KCOT is associated with syndromic and/or non-syndromic basal cell nevus.Case Report: The aim of the study was the clinico-pathological evaluation and comparison of two Iranian patients with syndromic and non-syndromic multiple KCOT.Conclusion: The syndromic multiple KCOT has a more aggressive behavior than single and non-syndromic types. Multiple KCOT might be the first sign of basal cell syndrome that is detected by a dentist. Evaluation of English papers showed that non-syndromic and syndromic types were the second and 20th case reports in the literature in the Iranian population, respectively.Key words: Basal cell nevus syndrome, Keratocystic odontogenic tumor, Non-syndromic, Syndromic.