Objective:Is to highlight the characteristics and management of odontogenic keratocyst in children onlyMaterial and Method:Computerized search in pubmed between (2005-2015) using specific words such as odontogenic keratocyst in children, odontogenic keratocyst association with Gorlin-Goltz syndrome with abstract written in English only.Result:During computerized literature search 77 articles in the years (2005-2015) were found. All these publications were miscellaneous studies including case series and case reports. Only 35 papers were selected which conform to our criteria. Most of the papers indicate that the histological type of keratocyst prevalent in children was parakeratinized variant, and most of the cases occurred in maxilla rather than mandible.Conclusion:We recommend that the surgeons who treat keratocysts in children take into consideration the late presentation in addition to the destructive nature and high recurrence rate. General practitioners face difficulty in early detection and referral of children with keratocysts or Gorlin syndrome. Treatment by multidiscplinary team is important if associated with Gorlin's syndrome Postoperative follow up is advised every 6 months.