en Gamma Knife Radiosurgery Case report <p style="text-align: justify;"><strong>Background & Importance:</strong> Iniencephaly is a rare cranio-cervical deformity. It belongs to neural tube defects. This disorder is&nbsp;characterized by a retroflexion of head, a very short neck, varying degrees of incomplete posterior closure of vertebrae and cervical and&nbsp;thoracic spinal synostosis. This malformation is associated with central nervous system and systemic malformations. Most of patients are&nbsp;dead born and others die after few hours of life. There are only few long-term survivors reported in literature.</p> <p style="text-align: justify;"><br> <strong>Case Presentation: </strong>A seven-year-old girl with iniencephaly signs who has been managed conservatively and still alive after six years of&nbsp;follow-up.</p> <p style="text-align: justify;"><br> <strong>Conclusion</strong>: Iniencephaly is a rare complexe disorder with a dismal prognosis but not uniformly fatal.</p> Iniencephaly, Neural Tube Defect, Spine 19 21 http://irjns.org/browse.php?a_code=A-10-57-4&slc_lang=en&sid=1 Souad Bakhti سوآد بختی 10031947532846003149 10031947532846003149 Yes Professor, Pediatric Neurosurgery Division, Mustapha Pacha University Hospital, Algiers, Algeria Nabila Tighilt نبیلا تیقلیت 10031947532846003150 10031947532846003150 No Assistant Professor, Pediatric Neurosurgery Division, Mustapha Pacha University Hospital, Algiers, Algeria Wahiba Khoudir واحیلا خودیر 10031947532846003151 10031947532846003151 No Consultant in Neurosurgery, Department of Neurosurgery, Mustapha Pacha University Hospital, Algiers, Algeria Mohamed Djennas محمد دیجناس 10031947532846003152 10031947532846003152 No Professor, Chief Department of Neurosurgery, Mustapha Pacha University Hospital, Algiers, Algeria