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Introduction: Encephalocele, midline defect of cranial bone fusion, occurs most frequently in the occipital region. Airway management in pediatric patients with craniofacial disorders poses many challenges to the anesthesiologist. The purpose of this study is to describe the airway problems encountered for such cases, and describe how these problems were managed. Materials and Methods: We reviewed the charts of occipital encephalocele newborn that were treated by surgical correction in Harran University Hospital during 2006-2008. The collected data were categorized into preoperative, intraoperative, and postoperative data. Results: The mean age of the patients was 5.17 days. Of these 17 patients, eight patients (47.1%) had hydrocephaly, one patient (5.8%) with Dandy Walker syndrome. Micrognathia, macroglossia, restriction in neck movements were recorded as the reasons in six cases each. No major anesthetic complication was found. Conclusions: We reported perioperative management in 17 occipital encephalocele infant. Comprehensive care during peroperative period is essential for successful outcome. [ABSTRACT FROM AUTHOR]